Treatment with Physical training
Uitgangsvraag
What is the effect of physical training in idiopathic inflammatory myopathy (IIM), IBM and JDM?
at is het effect van fysieke training bij idiopathische inflammatoire myopathie (IIM), IBM en JDM
Aanbeveling
Voor patiënten met een vorm van IIM (myositis) in het algemeen
Personaliseer de fysiotherapie.
Overweeg het trainingsprogramma af te stemmen met een centrum met voldoende expertise of een van de Revalidatieteams Neuromusculaire Ziekten.
Voor specifieke patiëntgroepen
Patiënten met myositis (exclusief IBM en JDM)
In het vroege stadium: Adviseer de patiënt te blijven bewegen, op geleide van klachten/spierzwakte en binnen de grenzen van zijn/haar kunnen.
Na het begin van de behandeling en/of bij tekenen van herstel: Overweeg kracht- en duurtraining, met een opbouw op geleide van klachten/spierzwakte.
Bij klinisch stabiele ziekteactiviteit: Overweeg intensievere kracht- en duurtraining vanwege een positief effect op aerobe capaciteit, spierkracht, spierprestaties en lichamelijke beperkingen.
Patiënten met JDM
Overweeg kracht- en duurtraining bij patiënten met een klinisch stabiele ziekteactiviteit aangezien is aangetoond dat dit veilig is en een positief effect heeft op spierpijn en vermoeidheid.
Patiënten met IBM
Overweeg kracht- en duurtraining bij patiënten met IBM in een vroeg ziektestadium omdat is aangetoond dat dit veilig is en een positief effectief heeft op de kwaliteit van leven.
Overweeg naarmate de ziekte vordert een overgang naar minder intensieve en passieve vormen van lichaamsbeweging.
Overwegingen
Considerations – from evidence to recommendation.
Pros and cons of the intervention and the quality of the evidence
A literature review was performed on the effectiveness of physical training (possibly in combination with drug treatment) in patients with (poly)myositis, juvenile dermatomyositis or inclusion body myositis. The functional outcomes aerobic capacity, muscle performance, fatigue, (muscle) pain and the outcome disability and quality of life were defined as critical outcomes. Muscle strength, disease activity, and disease damage were defined as critical outcomes.
The guideline development group considered aerobic capacity, muscle performance, fatigue, (muscle) pain, disability, and quality of life as critical outcomes for decision making; and muscle strength, disease activity, and disease damage as important outcomes for decision making.
Polymyositis and dermatomyositis
Based on the literature review, it can be concluded that the evidence is uncertain about the effect of physical training in patients with PM and DM on the crucial outcomes that fall under functional outcomes (aerobic capacity, muscle performance) and disability. A very low level of evidence was found for these outcomes. None of the included studies reported results for these patient groups for the crucial outcome measures fatigue and muscle pain. Therefore, it is not possible to make a statement about the effect of physical training in PM and DM for these outcomes. The crucial outcome measure quality of life was reported in the included studies, but based on the study results physical training seems to result in little or no difference on quality of life in patients with PM and DM compared to patients not undergoing physical training. For this, the level of evidence is low. Of the important outcome measures, information is only available on the outcome muscle strength, but again the literature is very uncertain about the effect of physical training on muscle strength in this patient group. The important outcomes disease activity and disease damage were not described.
Juvenile dermatomyositis
For JDM it seems that patients undergoing physical training experience less fatigue and muscle pain than patients not undergoing physical training. However, the level of evidence is low. In contrast, it is unclear whether physical training leads to increased aerobic capacity, muscle strength, and muscle performance. No conclusions could be drawn about the effect of physical training on disease activity, disease damage, disability, nor on quality of life.
Inclusion body myositis
For IBM, no conclusions could be drawn about the effect of physical training on aerobic capacity, muscle strength, muscle performance, fatigue, disease activity, disease damage, and disability. Physical training does seem to improve quality of life in patients with IBM. However, the level of evidence for this is low.
Patient values and preferences
In 2022, the Dutch myositis working group (Spierziekten Nederland) conducted a survey among patients with all types of IIM, in which they were asked about their perceptions and experiences regarding physical therapy and exercise programs. In total, fifteen patients participated in the survey. However, none of these patients were diagnosed with juvenile dermatomyositis.
The benefits and drawbacks of PT and exercise.
IIM and IBM patients were asked whether they found physical therapy and/or exercise a useful part of their treatment. All patients unanimously answered in the affirmative to this question. Patients reported benefits from personalized exercise programs for which they are dependent on a physical therapist. Many patients reported that during the first pandemic and the lockdown of COVID-19, when PT practices and gyms/fitness centers were closed, they experienced how dependent they were on the routine of exercise and PT treatment; many deteriorated, fell more, had more pain, or developed flare-ups of symptoms.
Benefits: possibilities of personalized exercise programs, feeling fit and well, both physically and mentally, the socializing aspect (meeting other people, getting out of the house), motivating when progress is seen, feeling better after exercise, the perception that one is actually, to some extent, taking/having control of one's body and the disease itself. Although not mentioned as goals per se, people with IIM (other than IBM) reported less pain and fatigue from exercise.
Drawbacks: Patients are dependent on the expertise and presence of PT/staff, it is sometimes difficult and tiring to get to or from locations. Patients find it difficult to assess their physical limits during exercise and wonder if this is harmful or not, moreover, an exercise program can become boring over time.
Reasons to consult the PT
IBM patients mostly consulted the PT for their exercise expertise, to get a personalized program. Not many of them went to the PT for treatment unlike the patients with IIM (DM/PM/ASyS/IMNM) who consulted PTs for two reasons: a personalized exercise program and treatment. Treatments were aimed at reducing pain and maintaining flexibility. The most frequently mentioned types of treatment were massage (muscles, tendons, joints), muscle stretching, breathing exercises, and relaxation exercises.
Goals of physical therapy and/or exercise
Patients with IBM gave the following top five answers: (I) prevention of deterioration; (II) improvement of endurance; (III) prevention of falling/improving balance; (IV) improvement of mobility; and (V) maintaining or improvement of mental health.
Patients with IIM reported some corresponding goals, but also some different ones compared to patients with IBM. They reported (I) improvement of muscle strength (mainly arms, legs, and core muscles); (II) improvement of endurance; (III) decreasing feelings of fatigue; (IV) improvement of flexibility of joints and muscles; and (V) maintaining or improvement of mental health important goals regarding physical therapy/exercise.
Type of exercise program
Patients with IIM preferred a combination of power- and endurance training, since focusing on only one of these exercise parameters was considered as tiring and boring. Patients with IBM, in contrast, preferred a combination of endurance and balance exercises. In the IBM population, no one mentioned hydrotherapy as a preferred exercise, a notable difference from many patients with other types of IIM who explicitly consider hydrotherapy to be a highly beneficial and highly valued part of their exercise routine. The main disadvantages reported were that dressing afterwards is tiring and that it is expensive (not reimbursed by health insurance).
Preferred exercise location and supervision
All IBM and IIM patients had consulted a physical therapist to get a personalized exercise program. All patients answered that they preferred a combination: many patients preferred to exercise at home (yoga, chair bike or treadmill, balance, in bed) or from home (mostly walking and cycling). The swimming pool (preferably warm water) and the PT practice are also popular, but depending on accessibility (stairs or elevator, parking options etc). The PT practice was preferred because of the presence of trained staff. The gym (fitness centre) was the least popular.
Currently, the IMACS (international myositis assessment and clinical studies group) is conducting a study called "rehabilitation and exercise in myositis" with the aim of developing recommendations and/or guidelines for exercises specified for each type of IIM. At the time of writing this article, focus groups are being held and so far the results of this small Dutch study are consistent with the results of the international group of DM/ PM, ASyS and IMNM patients.
Costs
There is no evidence on the cost-effectiveness of training in IIM. We can only assume that positive effects of training in IIM patients on functional outcomes, disability and QoL could lead to a higher level of physical activity, an increase in social participation, leading to lower social costs. Compared to other neurological diseases, neuromuscular diseases in general have the highest estimated cost of €30,000 per person per year, with an estimated prevalence between 1:20,000 to 1:424 persons (Gustavsson, 2010). These include direct health costs associated with treatment and rehabilitation, indirect costs associated with low labor force participation due to work absenteeism or early retirement, and non-medical costs for social services, assistive devices and informal care.
Acceptability, feasibility and implementation
Feasibility of the training in JDM was assessed by examining tolerability and adherence to the exercise intervention (Habers, 2016): Two patients were unable to start the intervention after the 12 weeks of usual care as a consequence of a disease relapse that occurred during the waiting control period. Of the remaining 24 patients, six patients (25%) started the intervention and stopped prematurely as a consequence of: lack of motivation/fatigue (n = 3; after 3, 7 and 11 sessions); recurrent infections (n = 1; after 9 sessions) and increasing complaints at the heel or knee (n = 2; both after 16 sessions). The other 18 patients (75%) completed the intervention and performed a median of 30 (interquartile range: 27–31) of the 32 sessions. Reasons for missing some of the sessions in this latter group included other sport activities, holiday, fatigue, illness, and transient physical complaints. Feasibility in DM, PM and IBM included studies (drop-out rate, percentage, and intensity of training) were not reported.
Moral and ethical concerns: training could be burdensome for patients with IIM, while there is only low evidence for the effectiveness of physical training.
Health equity: Reimbursement of physical therapy for neuromuscular diseases falls under the list of chronic diseases (formerly "Chronic list Borst"); physical therapy treatments are usually reimbursed (on referral of a specialist) from the 21st appointment onwards from the basic insurance, however, patients still often report problems in getting reimbursed for the physical therapy costs.
Conditions for feasibility and barriers to implementation: Patients depend on the expertise and presence of a PT/staff, expertise among health care providers is not always sufficient. Physical therapists from the Myositis Expertise Center or from one of the Neuromuscular Disease Rehabilitation Teams usually do, but physical therapists outside of these centers are not trained in this rare disease and need to receive specialized training. For patients, accessibility to the training site is poor or patients find it too stressful to get there.
Recommendations
This literature review shows that there is a knowledge gap in terms of training in IIM, IBM, and JDM, indicating the need for larger multicenter studies. However, given the rarity and the heterogeneity of the disease, large-scale international studies are difficult to conduct.
Polymyositis and dermatomyositis
For physical training, the evidence shows with very low certainty that there may be a slight increase in muscle performance and a slight decrease in disability for people with dermatomyositis and polymyositis. All training outcomes were in favor of the intervention group, although those related to aerobic capacity and muscle strength, were not clinically relevant. Physical training likely results in little to no difference in quality of life compared with no training in patients with polymyositis and dermatomyositis. Adverse side effects of training remained unclear, as well as the optimal training modality and intensity for PM and DM.
PM and DM patients consult a PT to benefit from a personalized exercise program and for treatments aimed at reducing pain and maintaining flexibility (massage, muscle stretching, breathing exercises, and relaxation exercises). Patients prefer an exercise program that includes a combination of strength and endurance exercises, since focusing on only one of these exercise parameters was considered as tiring and boring.
Because disease activity is not reported in the included PM and DM studies, the current training recommendations cannot be explored to patients with active inflammation early in the disease course. Safety of training (adverse events reflected by CK elevation, intensification of immunosuppression or hospitalization) and feasibility of training (drop-out rate, percentage and intensity of training) were also not reported in PM and DM studies.
The working group recommends to consider a combination of strength and endurance training in PM and DM patients with clinically stable disease, based on the a) positive effects of training, mostly clinically relevant, in favor of the training groups in the above-mentioned RCTs b) the ethical and methodological limitations associated with randomized and placebo-controlled trials to better investigate the effect of training in PM and DM and c) the unanimous response from PM and DM patients that they consider physical training a valuable treatment for their disease, with perceived health benefits and with goals (muscle strength, endurance, fatigue, flexibility of joints and muscles, mental health) largely consistent with the positive results of the studies.
Juvenile dermatomyositis
For physical training, there is evidence with very low certainty for a slight increase in aerobic capacity and muscle strength in patients with juvenile dermatomyositis. Physical training likely results in a decrease in fatigue and pain compared to no training in patients with JDM. These results are based on a single study, with a prescribed training modality and intensity (an individually tailored 12-week home exercise program, 2-3 times per week, of treadmill interval training and strength exercises), which was demonstrated safe in patients with clinically stable disease. The current training recommendations cannot be explored to patients with active inflammation.
The working group recommends to consider a combination of strength and endurance training in JDM patients with clinically stable disease, based on a) positive effects of training, mostly clinically relevant, in favor of the training groups in the above-mentioned RCT; b) the ethical and methodological limitations associated with randomized and placebo-controlled trials to better investigate the effect of physical training in JDM; and c) although the results are based on a single RCT, the generalizability of the findings is high because this RCT assessed eligibility for three quarters of patients with JDM with clinically stable disease in the Netherlands.
Inclusion body myositis
For physical training in IBM, the evidence shows with very low certainty that there were no clinically relevant outcomes in terms of aerobic capacity, muscle strength and fatigue. However, physical training probably does lead to a better quality of life in patients with IBM compared to no training in patients with IBM. IBM patients exhibit asymmetric weakness preferentially affecting the non-dominant hand (Dimachkie, 2013), suggesting that muscle strength could be trainable/maintainable, possibly justifying muscle strength training. Evidence with very low certainty indicates that training is safe, since there were no clinically relevant effects on disease activity or disease damage, as a result of the training. The optimal training modality and intensity for PM and DM is still unclear.
IBM patients mostly consulted the PT for their exercise expertise, to get a personalized program. IBM patients prefer a combination of endurance and balance exercises. The latter mainly for the purpose of reducing falls and maintaining mobility, no evidence was found for balance exercise. In patients with IBM, physical training can add value, but as the disease progresses, activities of daily living may already provide sufficient training stimulus or cause unwanted fatigue, making a gradual transition to lower intensity and passive exercise forms desirable. These forms of exercise are more focused on comfort and aim to reduce pain and contracture formation.
The working group recommends to consider physical training in IBM patients, in the early disease-stage, based on, a) positive effects of training, mostly clinically relevant, in favor of the training groups in the above-mentioned RCT, b) the ethical and methodological limitations associated with randomized and placebo-controlled trials to better investigate the effect of physical training in IBM, c) no negative side effects of physical training d) the unanimous response of IBM patients that they consider physical training a valuable treatment.
Onderbouwing
Achtergrond
Muscle involvement is the cardinal symptom in idiopathic inflammatory myopathy (IIM, “myositis”), but involvement of extra-muscular organ systems is common, such as ILD (Interstitial lung disease) and skin rash in DM. Patients with PM and DM present with reduced skeletal muscle strength, but more so with reduced muscle performance (Alexanderson, 2018). Also, cortico-induced myopathy may occur in addition to the weakness caused by the disease itself. Reductions in aerobic capacity have been reported in patients with both adult and juvenile IIM (Wiesinger, 1998, Takken 2003), compared to matched controls. Prominent other clinical features are pain and fatigue.
IBM patients experience reduced physical function and an elevated incidence of falls and may increasingly rely on help from caregivers to perform activities of daily living. Ultimately, IBM patients demonstrate lower quality of life.
As current medical treatment has proven ineffective for IBM patients, there is a strong need to identify alternative treatment methods for these patients (Jørgenson, 2018). And although a majority of IIM patients respond favorably on immunosuppressive treatment, many develop also sustained disability and report a reduced quality of life compared to the general population, suggesting the need for additional interventions, such as physical exercise (Alexanderson, 2018).
Even though a physically active lifestyle is fundamental to maintain health in general, patients with IIM previously were discouraged from exercise (Munters, 2013), since it is feared that the use of inflamed tissue would further worsen the inflammatory process (Wiesinger, 1998). Nowadays, exercise (training) is increasingly utilized in the clinical management of patients with IIM because several studies have shown no increase in muscle damage or inflammation after training. Moreover, various studies in patients with adult and juvenile IIM indicate that exercise training enhances muscle strength, aerobic capacity and functional outcomes (Habers, 2016; Munters, 2013).
Bottleneck in the current physical therapy treatment is the lack of recommendations regarding the content of an exercise program (defined in type of training, frequency, duration and intensity) and the description of patient relevant outcome measures, in both adult and juvenile IIM. Also, it is not clear at what stage of the disease (related to disease activity) exercise is effective and safe.
In this module regarding physical therapy, we primarily focus on physical training in IIM. In addition, physical therapy can also be significant in encouraging an active lifestyle, learning to cope with fatigue, reducing pain, fitting/trying out assistive devices, fall prevention, maintaining and monitoring lung function and coordinative shoulder training. With regard to these subjects, we refer to the brochure “Physical therapy in adults with slow progressive muscle disease” from Spierziekten Nederland (Spierziekten Nederland/KNGF, 2020).
Conclusies
Polymyositis and dermatomyositis
1. Functional outcomes
1.1 Aerobic capacity
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on aerobic capacity when compared with no physical training in patients with polymyositis and dermatomyositis.
Sources: Voet (2019) (Alemo Munters, 2013; Alexanderson, 2014; Wiesinger, 1998). |
1.2 Muscle strength
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on muscle strength when compared with no physical training in patients with polymyositis and dermatomyositis.
Sources: Voet (2019) (Alemo Munters, 2013). |
1.3 Muscle performance
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on muscle performance when compared with no physical training in patients with polymyositis and dermatomyositis.
Sources: Voet (2019) (Alemo Munters, 2013). |
1.4 Fatigue, 1.5 (muscle) pain, 1.6 disease activity, 1.7 disease damage
|
No GRADE |
Because of a lack of data in the included studies of this guideline, it was not possible to draw any conclusions regarding the outcome measures fatigue, muscle pain, disease activity, and disease damage in patients with polymyositis or dermatomyositis.
Sources: - |
2 Disability
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on disability when compared with no physical training in patients with polymyositis and dermatomyositis.
Sources: Voet (2019) (Wiesinger, 1998). |
3. Quality of life
|
Low GRADE |
Physical training likely results in little to no difference in quality of life when compared with no training in patients with polymyositis and dermatomyositis.
Sources: Voet (2019) (Alemo Munters, 2013; Alexanderson, 2014). |
Juvenile dermatomyositis
1. Functional outcomes
1.1 aerobic capacity
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on physical function when compared with no physical training in patients with juvenile dermatomyositis.
Sources: Voet (2019) (Habers, 2016). |
1.2 Muscle strength
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on muscle strength when compared with no physical training in patients with juvenile dermatomyositis.
Sources: Voet (2019) (Habers, 2016). |
1.3 Muscle performance
|
No GRADE |
Because of a lack of data in the included studies of this guideline, it was not possible to draw any conclusions regarding the outcome measure muscle performance in patients with juvenile dermatomyositis.
Sources: - |
1.4 Fatigue
|
Low GRADE |
Physical training likely reduces fatigue when compared with no physical training in patients with juvenile dermatomyositis.
Sources: Voet (2019) (Habers, 2016). |
1.5 (Muscle) pain
|
Low GRADE |
Physical training likely reduces muscle pain in comparison with no physical training in patients with juvenile dermatomyositis.
Sources: Voet (2019) (Habers, 2016). |
1.6 disease activity, 1.7 disease damage, 2. disability, 3. quality of life
|
No GRADE |
Because of a lack of data in the included studies of this guideline, it was not possible to draw any conclusions regarding the outcome measure disability, disease activity, disease damage, disability, and quality of life in patients with juvenile dermatomyositis.
Sources: - |
Inclusion body myositis
1. Functional outcomes
1.1 aerobic capacity
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on aerobic capacity when compared with no physical training in patients with inclusion body myositis.
Sources: Wallace (2019). |
1.2 Muscle strength
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on muscle strength when compared with no physical training in patients with inclusion body myositis.
Sources: Jørgensen (2018). |
1.3 Muscle performance 1.5 (muscle) pain
|
No GRADE |
Because of a lack of data in the included studies of this guideline, it was not possible to draw any conclusions regarding the outcome measure muscle performance and muscle pain in patients with inclusion body myositis.
Sources: - |
1.4 Fatigue
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on fatigue when compared with no physical training in patients with inclusion body myositis.
Sources: Wallace (2019). |
1.5 Disease activity
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on disease activity when compared with no physical training in patients with inclusion body myositis.
Sources: Jørgensen (2018). |
1.6 Disease damage
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on disease damage when compared with no physical training in patients with inclusion body myositis.
Sources: Jørgensen (2018). |
2. disability
|
Very low GRADE |
The evidence was very uncertain about the effect of physical training on disability when compared with no physical training in patients with inclusion body myositis.
Sources: Jørgensen (2018); Wallace (2019). |
3. Quality of life
|
Low GRADE |
Physical training likely increases quality of life when compared with no physical training in patients with juvenile dermatomyositis.
Sources: Jørgensen (2018); Wallace (2019). |
Samenvatting literatuur
Description of studies
The systematic (Cochrane) review of Voet (2019) assessed the effects (benefits and harms) of strength training and aerobic exercise training in people with a muscle disease. Voet (2019) included RCTs, quasi-RCTs or cross-over RCTs that compared: (I) strength training with no training; (II) aerobic exercise training with no training; (III) combined strength training and aerobic exercise versus no training; (IV) trials that included participants with a well-described diagnosis of a muscle disease, such as inflammatory myopathies, metabolic myopathies, muscular dystrophies, or muscle diseases with myotonia. Studies that looked at strength training or aerobic exercise training for people in whom muscle weakness was not the primary feature, but might have been secondary to chronic renal insufficiency, chronic heart failure, renal or heart transplantation, corticosteroid use were excluded. Moreover, studies using a within-participant design, with the non-exercised limb as a control were also excluded. The study searched the Cochrane Neuromuscular Specialised Register via the Cochrane Register of Studies (until 16 November 2018), the Cochrane Central Register of Controlled Trials (CENTRAL) (until 16 November 2018), MEDLINE (from 1946 to 15 November 2018), EMBASE (from 1974 to 15 November 2018), World Health Organization International Clinical Trials Registry Platform (until 22 December 2018), and the US National Institute of Health Ongoing Trials Register (until 22 December 2018). They also searched the bibliographies of the trials identified and other reviews of the subject and contacted some of the authors in the field to clarify trial eligibility or to identify additional published and unpublished data. In total, fourteen studies, including 449 participants, were included in the study. For the purpose of this guideline, four of these trials, involving 80 participants, compared physical training with no training in patients with (poly)myositis, dermatomyositis, inclusion body myositis, or juvenile dermatomyositis and were eligible for inclusion (Alemo Munters, 2013; Alexanderson, 2014; Habers, 2016; Wiesinger, 1998). All four studies were parallel group randomized controlled trials. The risk of bias was assessed according to the guidance in the Cochrane Handbook for Systematic Reviews of Interventions. The reported outcomes in the study were muscle strength, aerobic capacity, timed-scored functional assessments of muscle performance, quality of life measures, pain, experienced fatigue, and adverse effects requiring withdrawal of the participant from the study.
|
Author(s) |
Total N |
Intervention |
Control |
|
Alemo Munters (2013) |
Intervention: N = 11 (N=5 PM; N=6 JDM) Control: N = 10 (N=4 PM; N=6 JDM) |
Exercise training
Type: Cycling exercises and muscular endurance exercises.
Intensity: Aerobic exercise: during the first weeks, the exercise intensity was gradually increased from 50% up to 70% of the participants’ individual VO2 max. Strength training: 30%-40% of 1RM.
Frequency: 3 times/week, twice a week at a physical therapy department, once a week at home.
Duration: Session: 1 h. Programme: 12 weeks. |
No training |
|
Alexanderson (2014) |
Intervention: N = 10 (N=5 PM; N=5 JDM) Control: N = 9 (N=5 PM; N=4 JDM) |
Exercise training
Type: Resistive home exercise programme.
Intensity: Strength training: step up exercise for warm-up, shoulder flexion and knee extension in a sitting position, hip flexion and abduction, pelvic lifts and sit-ups lying down. Each exercise in 10 repetitions bilaterally, the programme ended with stretching. Exercise intensity was prescribed individually.
Aerobic exercise: a 15-min walk at an intensity level of 50%-70% of participants' estimated maximal heart rate.
Frequency: 5 times per week.
Setting: The 1st 12 weeks at home, the second 12 weeks at home and/or at the gym.
Duration: Not described, but varied individually. |
No training |
|
Habers (2016) |
Intervention: N = 14 (JDM) Control: N = 12 (JDM) |
Aerobic exercise training
Type: Interval treadmill training, strength training, at home.
Intensity: Aerobic training: individualised work rate, 30 min leg exercise on an ergo cycle, 65%-90% of the peak heart rate alternated with short periods of low-intensity exercise (50%-60% of peak heart rate).
Frequency: 2-3 times per week, total of 32 training sessions.
Duration: Session: 40-60 minutes. Programme: 12 weeks. |
No training |
|
Wiesinger (1998) |
Intervention: N = 7 (N=2 PM; N=5 DM)
Control: N = 7 (N=2 PM; N=5 DM) |
Aerobic exercise
Type: Endurance bicycle training, endurance step aerobics.
Intensity: Bicycle training: 30 min, slowly increased on an individual basis. Resistance was increased until a heart rate of 60% of maximum. Step aerobics: 30 min
Frequency: During the first two weeks, twice weekly, during the remaining four weeks, three times weekly.
Duration: Session: 60 minutes. Programme: 6 weeks. |
No training |
The phase 2 trial of Wallace (2019) assessed the feasibility and effect of community-based aerobic exercise training for people with inclusion body myositis. In total, seventeen patients were randomized in two groups. The patients in the intervention group (n=9) performed a twelve-week exercise program. There are no specific guidelines for exercise training in neuromuscular diseases and therefore Wallace (2019) adapted the aerobic training protocols recommended by the American College of Sport Medicine, considered gold standard for aerobic exercise to maintain health and fitness. Participants exercised on a bicycle ergometer 3 times per week for 12 weeks (36 sessions) working towards a duration of 30 minutes. All had heart rate monitors to set training targets. Initially the target heart rate corresponded to 60% of VO2 peak. The intensity was progressively increased to 70% after 4 weeks and 80% after 8 weeks. Each exercise session began with a 5-minute warm-up on the bicycle ergometer and ended with a 5- to 10-minute cool-down period. Participants were encouraged to exercise on alternative days to allow time for recovery and reduce general orthopedic stress. The control group (n=8) was instructed to continue their normal, prestudy activity levels. The reported outcomes in the study were physical function (VO2 peak, disability, and fatigue), and quality of life.
The randomized controlled trial of Jørgensen (2018) investigated the effect of twelve weeks of low-load blood-flow restricted resistance training on self-reported and objective physical function and maximal muscle strength in patients with sporadic inclusion body myositis. In total, 22 patients were randomized in two equally distributed groups. The patients in the intervention group (n=11) performed unilateral blood-flow restricted resistance training for both legs. In short, the protocol consisted of 12 weeks of training, twice per week. The following exercises were performed unilaterally in three sets of 25 ‘repetition maximum’ (four sets from week 9): leg press, knee extension, knee flexion (introduced from week 4), calf raise, and dorsal flexion. A 100 mm wide inflatable pneumatic cuff connected to a computerized tourniquet system was used to provide vascular occlusion to the lower limb. The cuff pressure was maintained at 110 mmHg. All training sessions were supervised by the same exercise physiologist. The patients in the control group did not follow an exercise program. The reported outcomes in the study were self-reported physical function evaluated using the SF-36, objective measures of physical function (2-minute Walk Test, Timed up and Go, and 30-Second Chair Stand test), everyday function evaluated with the Inclusion Body Myositis Functional Rating Scale (IBMFRS), Myositis Disease Activity Assessment Tool (MDAAT), Patient and Physician Global Activity and Damage evaluated on a 100 mm visual analogue scale, Myositis Damage Index (MDI), creatine kinase, Health Assessment Questionnaire (HAQ), and Manual Muscle Test (MMT-8).
Results
Polymyositis and dermatomyositis
1. Functional outcomes
1.1 Aerobic capacity
The outcome change in aerobic capacity in patients with polymyositis and dermatomyositis was reported in three studies, retrieved from the systematic review of Voet (2019) (Alemo Munters, 2013; Alexanderson, 2014; Wiesinger, 1998). Alemo Munters (2013) measured VO2 max during an exhaustion incremental test on a cycle ergometer, defined as the highest O2 uptake rate measured during the test. Alexanderson (2014) noninvasively estimated aerobic capacity using an eight-minute submaximal treadmill test. Wiesinger (1998) measured aerobic capacity during an incremental cycle test on a cycle ergometer, defining maximal oxygen uptake (VO2max) as the highest oxygen consumption obtained during the symptom-limited exercise test.
The mean (SD) change in physical function (VO2 max) in the study of Alemo Munters (2013) in the intervention group was 0.2 (0.1) milliliter per minute per kilogram, compared to -0.1 (5.0) milliliter per minute per kilogram in the control group. This resulted in a mean difference (MD) of 0.30 milliliter per minute per kilogram (95% CI -2.80 to 3.40), in favor of the intervention group. This was not considered as a clinically relevant difference.
The mean (SD) change in aerobic capacity (VO2 max) in the study of Alexanderson (2014) in the intervention group was 8.3 (5.2) milliliter per minute per kilogram, compared to 5.2 (6.5) milliliter per minute per kilogram in the control group. This resulted in a mean difference (MD) of 18.00 milliliter per minute per kilogram (95% CI 15.00 to 21.00), in favor of the intervention group. This was considered as a clinically relevant difference.
The mean (SD) change in aerobic capacity (highest oxygen uptake) in the study of Wiesinger (1998) in the intervention group was 12.0 (12.4) milliliter per minute per kilogram, compared to -2.6 (16.9) milliliter per minute per kilogram in the control group. This resulted in a mean difference (MD) of 14.60 milliliter per minute per kilogram (95% CI -0.93 to 30.13), in favor of the intervention group. This was not considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome aerobic capacity is derived from randomized controlled trials and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding of personnel in the study (risk of bias, -1), the small number of patients in the study (imprecision, -1). Consequently, the level of evidence is very low.
1.2 Muscle strength
The outcome change in muscle strength in patients with polymyositis and dermatomyositis was reported in one study, retrieved from the systematic review of Voet (2019) (Alemo Munters, 2013). Muscle strength was measured with manual muscle testing in eight muscle groups (MMT-8) (maximal isometric strength of neck flexors, middle deltoid, gluteus maximus, gluteus medius, biceps brachii, wrist extensors, wrist flexors, ankle dorsiflexors, on a scale from 0 (no movement) to 80 (normal) and by measuring the voluntary repetition maximum in knee the left and right extensors. The voluntary repetition maximum assessed the maximum load that a participant can lift in a full range of motion in five repetitions.
The mean (SD) change in MMT-8 score in the study of Alemo Munters (2013) in the intervention group was 4 (1.5), compared to 3 (3) in the control group. This resulted in a mean difference (MD) of 1.00 (95% CI -1.06 to 3.06), in favor of the intervention group. This was not considered as a clinically relevant difference.
The mean (SD) change in voluntary repetition maximum of the right knee extensors in the study of Alemo Munters (2013) in the intervention group was 3.8 (0.9), compared to 1.3 (0.9) in the control group. This resulted in a mean difference (MD) of 2.50 (95% CI 1.45 to 3.55), in favor of the intervention group. This was not considered as a clinically relevant difference.
The mean (SD) change in voluntary repetition maximum of the left knee extensors in the study of Alemo Munters (2013) in the intervention group was 3.8 (1.1), compared to 1.1 (0.7) in the control group. This resulted in a mean difference (MD) of 2.70 (95% CI 1.92 to 3.48), in favor of the intervention group. This was considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome muscle strength is derived from randomized controlled trials and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding of personnel in the study (risk of bias, -1) and the small number of patients in the study (imprecision, -2). Consequently, the level of evidence is very low.
1.3 Muscle performance
The outcome change in muscle performance in patients with polymyositis and dermatomyositis was reported in one study, retrieved from the systematic review of Voet (2019) (Alemo Munters, 2013). Muscle performance was assessed with the disease-specific Functional Index. The Functional Index Test includes testing of repetitions in eleven muscle groups: elbow flexion, shoulder flexion and abduction, hip flexion and abduction, step test, heel and toe lifts, neck flexion, and trunk flexion, with additional tests of ability to transfer from side to side lying down, transfer up to sitting, and peak expiratory flow.
The mean (SD) change in Functional Index score in the study of Alemo Munters (2013) in the intervention group was 17.1 (10.2), compared to 11.6 (8.5) in the control group. This resulted in a mean difference (MD) of 5.50 (95% CI -2.91 to 13.91), in favor of the intervention group. This was considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome muscle performance comes from randomized controlled trials and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding of personnel in the study (risk of bias, -1), the small number of patients, and the wide confidence interval crossing both boundaries of clinical relevance (both imprecision, -2). The level of evidence is very low.
1.4 Fatigue, 1.5. (muscle) pain, 1.6. disease activity, 1.7. disease damage
None of the included studies reported information regarding the outcomes fatigue, muscle pain, disease activity, and disease damage in patients with polymyositis and dermatomyositis.
Level of evidence of the literature
Due to a lack of relevant literature, it is not possible to perform a GRADE assessment for the level of evidence regarding the outcomes fatigue, (muscle) pain, disease activity, and disease damage in patients with polymyositis and dermatomyositis.
Level of evidence of the literature
The level of evidence regarding the outcome disability comes from randomized controlled trials and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding personnel in the study (risk of bias, -1), the small number of patients, and the wide confidence interval crossing both boundaries of clinical relevance (both imprecision, -2). Consequently, the level of evidence is very low.
2. Disability
The outcome change in disability in patients with polymyositis and dermatomyositis was reported in one study, retrieved from the systematic review of Voet (2019) (Wiesinger, 1998). Disability was measured with the Functional Assessment Screening Questionnaire. The Functional Assessment Screening Questionnaire (FASQ) is a 15-item checklist which was developed for primary care populations and may serve as a questionnaire method for evaluating disability which is associated with chronic pain.
The mean (SD) change in Functional Assessment Screening Questionnaire score in the study of Wiesinger (1998) in the intervention group was 20.5 (10.9), compared to 2.9 (29.3) in the control group. This resulted in a mean difference (MD) of 17.60 (95% CI -5.56 to 40.76), in favor of the intervention group. This was considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome disability comes from randomized controlled trials and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding of personnel in the study (risk of bias, -1), the small number of patients, and the wide confidence interval crossing both boundaries of clinical relevance (both imprecision, -2). The level of evidence is very low.
3. Quality of life
The outcome change in quality of life in patients with polymyositis and dermatomyositis was reported in two studies, retrieved from the systematic review of Voet (2019) (Alemo Munters, 2013; Alexanderson, 2014). Alemo Munters (2013) measured quality of life with the 36-item Short Form Health Survey (SF-36). All SF-36 scales range from 0 to 100 (optimal health). Alexanderson (2014) measured quality of life with the Notting Health Profile (NHP) Questionnaire. The NHP Questionnaire scores a range of possible scores from 0 (no perceived problems) to 100 (maximum problems) for six domains (energy, pain, sleep, social, emotional, and physical).
The mean (SD) change in SF-36 score for general health in the study of Alemo Munters (2013) in the intervention group was 15.5 (4.4), compared to 6.0 (4.9) in the control group. This resulted in a mean difference (MD) of 9.50 (95% CI 5.50 to 13.50), in favor of the intervention group. This was not considered as a clinically relevant difference.
The results for change in quality of life measured with the NHP Questionnaire in the study of Alexanderson (2014) for all eight domains are depicted in table 1:
Table 1: results NHP Questionnaire domains
|
Domain |
Intervention group, mean (SD) |
Control group, mean (SD) |
Mean difference (95% CI) |
Clinical relevant difference? |
|
Energy |
-21.1 (37.3) |
-3.1 (23.9) |
-18.00 (-45.90 to 9.90) |
Yes |
|
Pain |
-7.4 (14.7) |
-4.3 (0.1) |
-3.10 (-12.21 to 6.01) |
No |
|
Sleep |
-6.4 (27.1) |
-13.7 (6.1) |
7.30 (-9.96 to 24.56) |
No |
|
Social |
-4.3 (25.0) |
-5.4 (0.1) |
1.10 (-14.40 to 16.60) |
No |
|
Emotional |
-29.4 (29.1) |
-7.1 (9.7) |
-22.30 (-39.04 to -5.56) |
Yes |
|
Physical |
-10.3 (0.9) |
-8.5 (1.7) |
-1.80 (-3.04 to 0.56) |
No |
Level of evidence of the literature
The level of evidence regarding the outcome quality of life comes from randomized controlled trials and therefore starts high. The level of evidence was downgraded by two levels because of a lack of blinding of personnel in the study (risk of bias, -1) and the small number of patients in the study (imprecision, -1). Consequently, the level of evidence is low.
Juvenile dermatomyositis
1. Functional outcomes
1.1 Aerobic capacity
The outcome change in aerobic capacity in patients with juvenile dermatomyositis was reported in one study, retrieved from the systematic review of Voet (2019) (Habers, 2016). Habers (2016) defined aerobic capacity as endurance time: the time in minutes from the start to the end of a treadmill-based incremental maximal exercise test. Habers (2016) also reported the VO2 peak measured with a treadmill-based incremental maximal exercise test.
The mean (SD) change in endurance time in the study of Habers (2016) in the intervention group was -0.1 (0.4) minutes, compared to 1.1 (0.5) minutes in the control group. This resulted in a mean difference (MD) of -1.20 (95% CI -1.55 to -0.85), in favor of the intervention group. This was considered as a clinically relevant difference.
The mean (SD) change in VO2 oxygen peak in the study of Habers (2016) in the intervention group was 0 (1.4), compared to 2.1 (1.6) in the control group. This resulted in a mean difference (MD) of -2.10 (95% CI -3.27 to -0.93), in favor of the intervention group. This was considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome physical function comes from a randomized controlled trial and therefore starts high. The level of evidence was downgraded by two levels because of a lack of blinding of personnel in the study (risk of bias, -1), the confidence interval crossing the border of clinical relevance and the small number of patients in the study (both imprecision, -2). The level of evidence is very low.
1.2 Muscle strength
The outcome change in muscle strength in patients with juvenile dermatomyositis was reported in one study, retrieved from the systematic review of Voet (2019) (Habers, 2016). Habers (2016) measured the maximum force (Newton) for the knee extensors and the hip flexors for both left and right side.
The mean (SD) change in maximum force for the right knee extensors in the study of Habers (2016) in the intervention group was 31.0 (13.5) Newton, compared to -5 (15.0) (Newton) in the control group. This resulted in a mean difference (MD) of 36.00 (95% CI 24.95 to 47.05), in favor of the intervention group. This was considered as a clinically relevant difference.
The mean (SD) change in maximum force for the left knee extensors in the study of Habers (2016) in the intervention group was 13.0 (12.0) Newton, compared to -4 (27.0) (Newton) in the control group. This resulted in a mean difference (MD) of 17.00 (95% CI 0.48 to 33.52), in favor of the intervention group. This was considered as a clinically relevant difference.
The mean (SD) change in maximum force for the right hip flexors in the study of Habers (2016) in the intervention group was -4.0 (16.5) Newton, compared to 5 (18.0) (Newton) in the control group. This resulted in a mean difference (MD) of 9.00 (95% CI -22.36 to 4.36), in favor of the control group. This was considered as a clinically relevant difference.
The mean (SD) change in maximum force for the left hip flexors in the study of Habers (2016) in the intervention group was 9.0 (15.5) Newton, compared to 3 (17.0) (Newton) in the control group. This resulted in a mean difference (MD) of 6.00 (95% CI -6.59 to 18.59), in favor of the intervention group. This was not considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome muscle strength comes from a randomized controlled trial and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding of personnel in the study (risk of bias, -1), heterogeneity in the study results (inconsistency, -1), the wide confidence intervals crossing the boundaries of clinical relevance and the small number of patients in the study (both imprecision, -2). The level of evidence is very low.
1.3 Muscle performance
None of the included studies reported information regarding the outcome muscle performance in patients with juvenile dermatomyositis.
Level of evidence of the literature
Due to a lack of relevant literature, it is not possible to perform a GRADE assessment for the level of evidence regarding the outcome muscle performance in patients with juvenile dermatomyositis.
1.4 Fatigue
The outcome change in fatigue in patients with juvenile dermatomyositis was reported in one study, retrieved from the systematic review of Voet (2019) (Habers, 2016). Habers (2016) measured fatigue with the PedsQL Multidimensional Fatigue Scale (a scale from 0 to 100 on which higher scores indicate less fatigue).
The mean (SD) change in fatigue in the study of Habers (2016) in the intervention group was -1.0 (2.0), compared to 4.0 (2.0) in the control group. This resulted in a mean difference (MD) of -5.00 (95% CI -6.54 to -3.46), in favor of the intervention group. This was considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome fatigue comes from a randomized controlled trial and therefore starts high. The level of evidence was downgraded by two levels because of a lack of blinding of personnel in the study (risk of bias, -1) and the small number of patients in the study (imprecision, -1). The level of evidence is low.
1.5 Muscle pain
The outcome change in muscle pain in patients with juvenile dermatomyositis was reported in one study, retrieved from the systematic review of Voet (2019) (Habers, 2016). Habers (2016) measured muscle pain with the visual analogue scale (a scale from zero to 10 in which a higher score indicates more pain).
The mean (SD) change in muscle pain in the study of Habers (2016) in the intervention group was -3.0 (3.5), compared to 4.0 (3.5) in the control group. This resulted in a mean difference (MD) of -7.00 (95% CI -9.70 to -4.30), in favor of the intervention group. This was considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome muscle pain comes from a randomized controlled trial and therefore starts high. The level of evidence was downgraded by two levels because of a lack of blinding of personnel in the study (risk of bias, -1) and the small number of patients in the study (imprecision, -1). The level of evidence is low.
1.6. Disease activity, 1.7. disease damage, 2. Disability, 3. quality of life
None of the included studies reported information regarding the outcomes disease activity, disease damage, disability and quality of life in patients with juvenile dermatomyositis.
Level of evidence of the literature
Due to a lack of relevant literature, it is not possible to perform a GRADE assessment for the level of evidence regarding the outcome’s disease activity, disease damage, disability and quality of life in patients with juvenile dermatomyositis.
Inclusion body myositis
1. Functional outcomes
1.1 Aerobic capacity
The outcome aerobic capacity in patients with inclusion body myositis was reported in one study (Wallace, 2019).Wallace (2019) reported physical function measured with the six-minute walk test and reported the peak oxygen uptake (VO2 peak).
The mean (SD) distance of the six-minute walk test in the study of Wallace (2019) in the intervention group was 258.93 (66.73) meter, compared to 260.86 (83.55) meter in the control group. This resulted in a mean difference (MD) of -1.93 (-74.40 to 70.54) meter in favor of the control group. This was not considered as a clinically relevant difference.
The mean (SD) VO2 peak (milliliter per minute per kilogram) in the study of Wallace (2019) in the intervention group was 15.71 (3.35) mL/min/kg, compared to 13.29 (2.63) mL/min/kg in the control group. This resulted in a mean difference (MD) of 2.42 (95% CI -0.43 to 5.27), in favor of the intervention group. This was not considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome aerobic capacity comes from a randomized controlled trial and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding of personnel in the study (risk of bias, -1), heterogeneity in the study results (inconsistency, -1), the wide confidence intervals crossing the boundaries of clinical relevance, and the small number of patients in the study (both imprecision, -2). The level of evidence is very low.
1.2 Muscle strength
The outcome muscle strength was reported in one study (Jørgensen, 2018). Muscle strength was measured with manual muscle testing in eight muscle groups (MMT-8) (maximal isometric strength of neck flexors, middle deltoid, gluteus maximus, gluteus medius, biceps brachii, wrist extensors, wrist flexors, ankle dorsiflexors, on a scale from 0 (no movement) to 80 (normal)) and by measuring the knee extensor strength in newton meter per kilogram (Nm/kg).
The mean (SD) MMT-8 score in the study of Jørgensen (2018) in the intervention group was 71.2 (5.4), compared to 66.9 (6.1) in the control group. This resulted in a mean difference (MD) of 4.30 (-0.51 to 9.11), in favor of the intervention group. This was not considered as a clinically relevant difference.
The mean (SD) knee extensor strength in the study of Jørgensen (2018) in the intervention group was 0.62 (0.55) Nm/kg, compared to 0.53 (0.50) in the control group. This resulted in a mean difference (MD) of 0.09 (95% CI -0.35 to 0.53), in favor of the intervention group. This was not considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome muscle strength comes from a randomized controlled trial and therefore starts high. The level of evidence was downgraded by … levels because of a lack of blinding of personnel in the study (risk of bias, -1), the wide confidence interval crossing the boundaries of clinical relevance, and the small number of patients in the study (both imprecision, -2). The level of evidence is very low.
1.3 Muscle performance, 1.4 muscle pain
None of the included studies reported information regarding the outcomes muscle performance and muscle pain in patients with inclusion body myositis.
Level of evidence of the literature
Due to a lack of relevant literature, it is not possible to perform a GRADE assessment for the level of evidence regarding the outcomes muscle performance and muscle pain in patients with inclusion body myositis.
1.5 fatigue
The outcome change in fatigue in patients with inclusion body myositis was reported in one study (Wallace, 2019). Wallace (2019) measured fatigue with the Fatigue Severity Scale (FFS). The questionnaire contains nine questions (scores ranging from 1 (completely disagree) to 7 (in full agreement) to assess the perceived severity of fatigue symptoms in the past week in various daily situations. The patient indicates the extent to which the fatigue determines the functioning.
The mean (SD) FFS score in the study of Wallace (2019) in the intervention group was 29 (26), compared to 29 (18) in the control group. This resulted in a mean difference (MD) of 0.00 (95% CI -21.07 to 21.07), not in favor of one of the groups. This was not considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome fatigue comes from a randomized controlled trial and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding of personnel in the study (risk of bias, -1), the wide confidence interval crossing the boundaries of clinical relevance, and the small number of patients in the study (both imprecision, -2). The level of evidence is very low.
1.6 Disease activity
The outcome disease activity was reported in one study (Jørgensen, 2018). Disease activity was measured with the Myositis Disease Activity Tool (MDAAT) (a scale from 0 to 10 (extremely severe disease activity) that assesses six extra muscular organs to produce a global extra muscular score, and the muscle score, which gives a total disease activity index score) and the Patient Global Activity (a scale from 0 to 100 on which a higher score represents worse perceived disease activity or overall health).
The mean (SD) MDAAT score in the study of Jørgensen (2018) in the intervention group was 0.10 (0.07), compared to 0.18 (0.12) in the control group. This resulted in a mean difference (MD) of -0.08 (95% CI -0.16 to 0.00), in favor of the intervention group. This was not considered as a clinically relevant difference.
The mean (SD) Patient Global Activity score in the study of Jørgensen (2018) in the intervention group was 13.5 (25.9), compared to 19.0 (18.5) in the control group. This resulted in a mean difference (MD) of -5.50 (-24.31 to 13,31), in favor of the intervention group. This was not considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome disease activity comes from randomized controlled trials and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding of personnel in the study (risk of bias, -1), the wide confidence interval crossing the boundaries of clinical relevance, and the small number of patients in the study (both imprecision, -2). The level of evidence is very low.
1.7 Disease damage
The outcome measure disease damage was reported in one study (Jørgensen, 2018). Disease damage was measured with the Myositis Damage Index (MDI) which assessed the degree of damage of various organ systems on a 0 to 10 (most damage) scale.
The mean (SD) MDI score in the study of Jørgensen (2018) in the intervention group was 0.19 (0.06), compared to 0.17 (0.07) in the control group. This resulted in a mean difference (MD) of 0.02 (95% CI -0.03 to 0.07), in favor of the control group. This was not considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome disease damage comes from randomized controlled trials and therefore starts high. The level of evidence was downgraded by three levels because of a lack of blinding of personnel in the study (risk of bias, -1), the wide confidence interval crossing the boundaries of clinical relevance, and the small number of patients in the study (both imprecision, -2). The level of evidence is very low.
2. Disability
The outcome disability in patients with inclusion body myositis was reported in two studies (Jørgensen, 2018; Wallace, 2019). Jørgensen (2018) reported outcomes on the Inclusion Body Myositis Functional Rating Scale (IBMFRS) (a scale from 0 to 40 on which a higher score means less disease severity) and the Health Assessment Questionnaire (HAQ) which measures limitation in performing activity of general daily living. Wallace (2019) only reported disability measured with the IBMFRS.
The mean (SD) IBMFRS score in the study of Jørgensen (2018) in the intervention group was 32.5 (4.9), compared to 29.7 (4.9) in the control group. This resulted in a mean difference (MD) of 2.80 (95% CI -1.30 to 6.90), in favor of the intervention group. This was not considered as a clinically relevant difference.
The mean (SD) IBMFRS score in the study of Wallace (2019) in the intervention group was 25 (11), compared to 25 (10) in the control group. This resulted in a mean difference (MD) of 0.00 (95% CI -9.98 to 9.98), not favoring one of the groups. This was not considered as a clinically relevant difference.
The mean (SD) HAQ score in the study of Jørgensen (2018) in the intervention group was 0.89 (0.73), compared to 1.02 (0.79) in the control group. This resulted in a mean difference (MD) of -0.13 (95% CI -0.77 to 0.51), in favor of the intervention group. This was not considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome disability comes from randomized controlled trials and therefore starts high. The level of evidence was downgraded by two levels because of a lack of blinding of personnel in the study (risk of bias, -1), the wide confidence intervals crossing the boundaries of clinical relevance, and the small number of patients in the study (both imprecision, -2). The level of evidence is very low.
3. Quality of life
The outcome quality of life in patients with inclusion body myositis was reported in two studies (Jørgensen, 2018; Wallace, 2019). Jørgensen (2018) measured quality of life with the 36-item Short Form Health Survey (SF-36). All SF-36 scales range from 0 to 100 (optimal health). Wallace (2019) also reported quality of life with the SF-36.
The mean (SD) SF-36 score for general health in the study of Jørgensen (2018) in the intervention group was 57.8 (17.6), compared to 32.3 (20.4) in the control group. This resulted in a mean difference (MD) of 25.50 (95% CI 9.58 to 41.42), in favor of the intervention group. This was considered as a clinically relevant difference.
The mean (SD) SF-36 score for in the study of Wallace (2019) in the intervention group was 92 (8), compared to 90 (10) in the control group. This resulted in a mean difference (MD) of 2.00 (95% CI -6.68 to 10.68), in favor of the intervention group. This was not considered as a clinically relevant difference.
Level of evidence of the literature
The level of evidence regarding the outcome quality of life comes from randomized controlled trials and therefore starts high. The level of evidence was downgraded by two levels because of a lack of blinding of personnel in the study (risk of bias, -1) and the small number of included patients (imprecision, -1). The level of evidence is low.
Zoeken en selecteren
A systematic review of the literature was performed to answer the following question:
What is the effect of physical training on functional outcomes, disability and quality of life in idiopathic inflammatory myopathy (IIM), IBM and JDM in addition to UC?
P: Patients with probable or diagnosed (poly)myositis, juvenile dermatomyositis, and inclusion body myositis.
I: Medicinal treatment with physical training (strength, fitness) / active lifestyle program / rehabilitation.
C: Medicinal treatment or usual care.
O: Disorder of body functions and structures, activities and participation, quality of life.
Relevant outcome measures
Exercise effects will be described based on the International Classification of Functioning Disability and Health (ICF) (WHO, 2001). A framework that describes health and health-related conditions as well as the impact of a health-related condition on the affected individual. According to the ICF, the exercise effects will be described in terms of:
Functional outcomes indicate the physiological functions of body systems and body structures, such as aerobic capacity, muscle performance, fatigue, (muscle) pain, disease activity and disease damage.
Disability is used an umbrella term for impairments, activity limitations and participation restrictions. It denotes the negative aspects of the interaction between a person’s health condition(s) and that individual’s contextual factors (environmental and personal factors).
Quality of life is defined as 'individuals' perceptions of their position in life in the context of the culture and value systems in which they live, and in relation to their goals, expectations, standards and concerns.
The guideline development group considered aerobic capacity, muscle performance, fatigue, (muscle) pain, disability and quality of life as critical outcomes for decision making; and muscle strength, disease activity, and disease damage as important outcomes for decision making.
A priori, the working group did not define the outcome listed above but used the definitions used in the studies.
The working group defined a threshold of 10% for continuous outcomes, such as physical function, disability, muscle performance, fatigue, muscle pain, disease activity, disease damage, and quality of life), a 15% threshold for the continuous outcome muscle strength, and 25% threshold in relative risk (RR) for dichotomous outcomes as a minimal clinically important difference.
Search and select (Methods)
The databases Medline (via OVID) and Embase (via Embase.com) were searched with relevant search terms until the 15th of July 2021. The detailed search strategy is depicted under the tab Methods. The systematic literature search resulted in 490 hits. Studies were selected based on the following criteria:
- Study design: systematic reviews and randomized controlled trials
- Study population: patients with IIM, juvenile dermatomyositis, or inclusion body myositis.
- Intervention: physical therapy
- Articles published in English or Dutch
- Articles published between 1993 and July 2021
- Full-text version or articles available
Thirty-one studies were initially selected based on title and abstract screening. After reading the full text, twenty-eight studies were excluded (see the table with reasons for exclusion under the tab Methods), and three studies were included (Voet, 2019; Wallace, 2019; Jørgensen, 2018)
Results
Three studies were included in the analysis of the literature. Important study characteristics and results are summarized in the evidence tables. The assessment of the risk of bias is summarized in the risk of bias tables.
Referenties
- Alemo Munters L, Dastmalchi M, Andgren V, Emilson C, Bergegård J, Regardt M, Johansson A, Orefelt Tholander I, Hanna B, Lidén M, Esbjörnsson M, Alexanderson H. Improvement in health and possible reduction in disease activity using endurance exercise in patients with established polymyositis and dermatomyositis: a multicenter randomized controlled trial with a 1-year open extension followup. Arthritis Care Res (Hoboken). 2013 Dec;65(12):1959-68. doi: 10.1002/acr.22068. PMID: 23861241.
- Alexanderson H, Munters LA, Dastmalchi M, Loell I, Heimbürger M, Opava CH, Lundberg IE. Resistive home exercise in patients with recent-onset polymyositis and dermatomyositis -- a randomized controlled single-blinded study with a 2-year followup. J Rheumatol. 2014 Jun;41(6):1124-32. doi: 10.3899/jrheum.131145. Epub 2014 May 1. PMID: 24786930.
- Dimachkie MM, Barohn RJ. Inclusion body myositis. Curr Neurol Neurosci Rep. 2013 Jan;13(1):321. doi: 10.1007/s11910-012-0321-4. PMID: 23250766.
- Gustavsson A, Svensson M, Jacobi F, Allgulander C, Alonso J, Beghi E, Dodel R, Ekman M, Faravelli C, Fratiglioni L, Gannon B, Jones DH, Jennum P, Jordanova A, Jönsson L, Karampampa K, Knapp M, Kobelt G, Kurth T, Lieb R, Linde M, Ljungcrantz C, Maercker A, Melin B, Moscarelli M, Musayev A, Norwood F, Preisig M, Pugliatti M, Rehm J, Salvador-Carulla L, Schlehofer B, Simon R, Steinhausen HC, Stovner LJ, Vallat JM, Van den Bergh P, van Os J, Vos P, Xu W, Wittchen HU, Jönsson B, Olesen J; CDBE2010Study Group. Cost of disorders of the brain in Europe 2010. Eur Neuropsychopharmacol. 2011 Oct;21(10):718-79. doi: 10.1016/j.euroneuro.2011.08.008. Epub 2011 Sep 15. Erratum in: Eur Neuropsychopharmacol. 2012 Mar;22(3):237-8. den Bergh, Peter Van (corrected to Van den Bergh, Peter). PMID: 21924589.
- Habers GE, Bos GJ, van Royen-Kerkhof A, Lelieveld OT, Armbrust W, Takken T, van Brussel M. Muscles in motion: a randomized controlled trial on the feasibility, safety and efficacy of an exercise training programme in children and adolescents with juvenile dermatomyositis. Rheumatology (Oxford). 2016 Jul;55(7):1251-62. doi: 10.1093/rheumatology/kew026. Epub 2016 Mar 27. PMID: 27018060.
- Jørgensen AN, Aagaard P, Frandsen U, Boyle E, Diederichsen LP. Blood-flow restricted resistance training in patients with sporadic inclusion body myositis: a randomized controlled trial. Scand J Rheumatol. 2018 Sep;47(5):400-409. doi: 10.1080/03009742.2017.1423109. Epub 2018 May 18. PMID: 29775118.
- Spierziekten Nederland/KNGF. Fysiotherapie bij volwassenen met een langzaam progressieve spierziekte. 2020.
- Voet NB, van der Kooi EL, van Engelen BG, Geurts AC. Strength training and aerobic exercise training for muscle disease. Cochrane Database Syst Rev. 2019 Dec 6;12(12):CD003907. doi: 10.1002/14651858.CD003907.pub5. PMID: 31808555; PMCID: PMC6953420.
- Wallace A, Pietrusz A, Dewar E, Dudziec M, Jones K, Hennis P, Sterr A, Baio G, Machado PM, Laurá M, Skorupinska I, Skorupinska M, Butcher K, Trenell M, Reilly MM, Hanna MG, Ramdharry GM. Community exercise is feasible for neuromuscular diseases and can improve aerobic capacity. Neurology. 2019 Apr 9;92(15):e1773-e1785. doi: 10.1212/WNL.0000000000007265. Epub 2019 Mar 8. PMID: 30850441; PMCID: PMC6511083.
- World Health Organization. (2001). International classification of functioning, disability and health : ICF. World Health Organization. https://apps.who.int/iris/handle/10665/42407
Evidence tabellen
|
Study reference |
Study characteristics |
Patient characteristics |
Intervention (I) |
Comparison / control (C) |
Follow-up |
Outcome measures and effect size |
Comments |
|
Voet (2019) |
SR and meta-analysis of RCTs, quasi-RCTs, or cross-over RCTs that made any of the following comparisons:
Literature search up to December 2018
Study design: RCT (parallel / cross-over), cohort (prospective / retrospective), case-control
Setting and Country:
Sources:
Internal sources • Department of Rehabilitation, Radboud University Medical Centre, Nijmegen, Netherlands. Salary; • Department of Neurology, Radboud University Medical Centre, Nijmegen, Netherlands. Salary; • Rehabilitation Centre Klimmendaal, Arnhem, Netherlands. Salary; • Department of Neurology, Medical Centre Leeuwarden, Leeuwarden, Netherlands. Salary
External sources; • No sources of support supplied
Conflict of interest: E van der Kooi carried out a randomised controlled trial (RCT) on the eMect of strength training and albuterol in facioscapulohumeral muscular dystrophy (FSHD; Van der Kooi 2004).
NBM Voet received grant support from the Netherlands Organisation for Health Research and Development (ZonMw), Princess Beatrix Muscle Fund (PBS), and the Dutch FSHD Foundation.
NBM Voet, BGM van Engelen and ACH Geurts carried out a RCT on the eMect of aerobic exercise in FSHD (Voet 2014).
BGM van Engelen was research director of the European Neuromuscular Centre and receives institutional support from the Radboud University Medical Centre and the ENMC, grant support from European Union’sHorizon 2020 research and innovation programme (Murab), European Union 7th Framework Programme (OPTIMISTIC), the Netherlands Organisation for Scientific Research (NWO), The Netherlands Organisation for Health Research and Development (ZonMw), Global FSH, Prinses Beatrix Fonds, Spieren voor Spieren, Association Francaise contre les Myopathies, and the Dutch FSHD Foundation. He is consultant and clinical advisor of Fulcrum.
ACH Geurts receives institutional support from the Radboud University Medical Centre, and grant support from the Netherlands Organisation for ScientificResearch (NWO), TheNetherlandsOrganisation forHealthResearch andDevelopment(ZonMw), PrincessBeatrix Muscle Fund (PBS), and the Dutch FSHD Foundation.
|
Inclusion criteria SR:
Exclusion criteria SR:
4 studies included
Important patient characteristics at baseline:
N
Age:
Sex M/F:
Groups comparable at baseline? Yes. |
Describe intervention:
|
Describe control:
|
End-point of follow-up:
For how many participants were no complete outcome data available? (intervention/control)
|
Aerobic exercise training versus no training in dermatomyositis and polymyositis
Aerobic capacity (mL/min/kg), mean (SD) Reported in one study (Wiesinger, 1998) Con: -2.6 (16.9) MD (95% CI): 14.60 (-0.93 to 30.13)
Functional assessment – Functional assessment Screening Questionnaire, mean (SD) Reported in one study (Wiesinger, 1998) Int: 20.5 (10.9) Con: 2.9 (29.3) MD (95% CI): 17.60 (-5.56 to 40.76)
Aerobic exercise and strength training versus no training in dermatomyositis and polymyositis
Manual muscle strength testing in 8 muscle groups (MMT-8), mean (SD) Reported in one study (Alemo Munters, 2013) Int: 4 (1.5) Con: 3 (3) MD (95% CI): 1.00 (-1.06 to 3.06)
Voluntary repetition maximum in knee extensors, right (kg), mean (SD) Reported in one study (Alemo Munters, 2013) Int: 3.8 (0.9) Con: 1.3 (0.9) MD (95% CI): 2.50 (1.45 to 3.55)
Voluntary repetition maximum in knee extensors, left (kg), mean (SD) Reported in one study (Alemo Munters, 2013) Int: 3.8 (1.1) Con: 1.1 (0.7) MD (95% CI): 2.70 (1.92 to 3.48)
Power performed at VO2 max (W), mean (SD) Reported in one study (Alemo Munters, 2013) Int: 14 (3.5) Con: -4 (3.5) MD (95% CI): 18.00 (15.00 to 21.00)
Time to exhaustion in endurance cycling test (min), mean (SD) Reported in one study (Alemo Munters, 2013) Int: 18.3 (13.3) Con: 0.8 (4.8) MD (95% CI): 17.50 (8.00 to 27.00)
VO2 max, mean (SD) Reported in two studies (Alexanderson, 2014; Alemo Munters, 2013)
Alexanderson (2014) Int: 8.3 (5.2) Con: 5.2 (6.5)
Alemo Munters (2013) Int: 0.2 (1.1) Con: -0.1 (5)
Pooled MD (95% CI): 1.03 (-1.69 to 3.75) (random effect measures), favoring the intervention group.
Disease-specific functional index 0-64 Reported in one study (Alexanderson, 2014) Int: 17.1 (10.2) Con: 11.6 (8.5) MD (95% CI): 5.50 (-2.91 to 13.91)
Quality of life – physical function Reported in two studies (Alexanderson, 2014; Alemo Munters, 2013)
Alexanderson (2014) Int: 10.3 (0.9) Con: 8.5 (1.7)
Alemo Munters (2013) Int: 8.9 (3.2) Con: -0.2 (6.6)
Pooled MD (95% CI): 5.12 (-2.01 to 12.24) (random effect measures), favoring the intervention group.
Quality of life – SF-36 General health 0-100 Reported in one study (Munters, 2013) Int: 15.5 (4.4) Con: 6 (4.9) MD (95% CI): 9.50 (5.50 to 13.50)
Quality of life – SF-36 Vitality 0-100) Reported in one study (Alemo Munters, 2013) Int: 11.8 (4.6) Con: -0.5 (5.1) MD (95% CI): 12.30 (8.13 to 16.47)
Quality of life – SF-36 Mental health 0-100) Reported in one study (Alemo Munters, 2013) Int: 8.1 (3.7) Con: 3.1 (4.1) MD (95% CI): 5.00 (1.65 to 8.35)
Quality of life – SF-36 NHP energy 0-100) Reported in one study (Alexanderson, 2014) Int: -21.1 (37.3) Con: -3.1 (23.9) MD (95% CI): -18.00 (-45.90 to 9.90)
Quality of life – SF-36 NHP pain 0-100) Reported in one study (Alexanderson, 2014) Int: -7.4 (14.7) Con: -4.3 (0.1) MD (95% CI): -3.10 (-12.21 to 6.01)
Quality of life – SF-36 NHP sleep 0-100) Reported in one study (Alexanderson, 2014) Int: -6.4 (27.1) Con: -13.7 (6.1) MD (95% CI): 7.30 (-9.96 to 24.56)
Quality of life – SF-36 NHP social 0-100) Reported in one study (Alexanderson, 2014) Int: -4.3 (25) Con: -5.4 (0.1) MD (95% CI): 1.10 (-14.40 to 16.60)
Quality of life – SF-36 NHP emotional 0-100) Reported in one study (Alexanderson, 2014) Int: -29.4 (29.1) Con: -7.1 (9.7) MD (95% CI): -22.30 (-39.04 to -5.56)
Quality of life – SF-36 NHP physical 0-100) Reported in one study (Alexanderson, 2014) Int: -10.3 (0.9) Con: -8.5 (1.7) MD (95% CI): -1.80 (-3.04 to -0.56)
Aerobic exercise and strength training versus no training in juvenile dermatomyositis
Max force right knee extensors (N), mean (SD) Reported in one study (Habers, 2016) Int: 31 (13.5) Con: -5 (15) MD (95% CI): 36.00 (24.95 to 47.05)
Max force left knee extensors (N), mean (SD) Reported in one study (Habers, 2016) Int: 13 (12) Con: -4 (27) MD (95% CI): 17.00 (0.48 to 33.52)
Max force right hip flexors (N), mean (SD) Reported in one study (Habers, 2016) Int: -4 (16.5) Con: 5 (18) MD (95% CI): -9.00 (-22.36 to 4.36)
Max force left hip flexors (N), mean (SD) Reported in one study (Habers, 2016) Int: 9 (15.5) Con: 3 (17) MD (95% CI): 6.00 (-6.59 to 18.59)
Endurance time during maximal exercise test (min), mean (SD) Reported in one study (Habers, 2016) Int: -0.1 (0.4) Con: 1.1 (0.5) MD (95% CI): -1.20 (-1.55 to -0.85)
VO2 peak (mL/kg/min), mean (SD) Reported in one study (Habers, 2016) Int: 0 (1.4) Con: 2.1 (1.6) MD (95% CI): -2.10 (-3.27 to -0.93)
Distance walked in 6-min walk test (m), mean (SD) Reported in one study (Habers, 2016) Int: 2 (17.5) Con: 9 (20) MD (95% CI): -7.00 (-21.56 to 7.56)
Muscle pain (10-cm VAS), mean (SD) Reported in one study (Habers, 2016) Int: -3 (3.5) Con: 4 (3.5) MD (95% CI): -7.00 (-9.70 to -4.30)
Perception of fatigue – PedsQL Multidimensional fatigue scale 0-100), mean (SD) Int: -1 (2) Con: 4 (2) MD (95% CI): -5.00 (-6.54 to -3.46) |
Authors’ conclusion:
The evidence regarding strength training and aerobic exercise interventions remains uncertain. Evidence suggests that strength training alone may have little or no eMect, and that aerobic exercise training alone may lead to a possible improvement in aerobic capacity, but only for participants with FSHD. For combined aerobic exercise and strength training, there may be slight increases in muscle strength and aerobic capacity for people with dermatomyositis and polymyositis, and a slight decrease in aerobic capacity and increase in muscle strength for people with juvenile dermatomyositis. More research with robust methodology and greater numbers of participants is still required.
|
|
Study reference |
Study characteristics |
Patient characteristics |
Intervention (I) |
Comparison / control (C)
|
Follow-up |
Outcome measures and effect size |
Comments |
|
Jørgensen (2018) |
Type of study: Randomized controlled trial.
Setting and country: Odense University Hospital and at the University of Southern Denmark, Denmark.
Funding: This work was funded by the Region of Southern Denmark (2012 j. nr. 12/7763), the Danish Rheumatism Association (R108-A2413) and Danish Rheumatism Association (SE no. 2928 3958), and the AP Møller Foundation for the Advancement of Medical Science (SE no. 2928 3958).
Conflicts of interest: Not reported.
|
Inclusion criteria:
Exclusion criteria:
N total at baseline: Intervention: N = 11 Control: N = 11
Important prognostic factors: age ± SD: I: 68.1 (6.4) C: 69.8 (4.8)
Sex: I: 9/11 (82%) M C: 9/11 (82%) M
Groups comparable at baseline? Yes.
|
Describe intervention (treatment/procedure/test):
The BFR training protocol was consisted of 12 weeks of training, twice per week. The following exercises were performed unilaterally in three sets of 25 ‘repetition maximum’ (four sets from week 9): leg press, knee extension, knee flexion (introduced from week 4), calf raise, and dorsal flexion. A 100 mm wide inflatable pneumatic cuff connected to a computerized tourniquet system was used to provide vascular occlusion to the lower limb. The cuff pressure was maintained at 110 mmHg. All training sessions were supervised by the same exercise physiologist.
|
Describe control (treatment/ procedure/test):
No-exercise control group. |
Length of follow-up: 12 weeks.
Loss-to-follow-up: None.
Incomplete outcome data: None.
|
Physical function – SF-36 (0 (worst) to 100), mean (SD) (95% CI) I: 57.8 (17.6) (95% CI 47.4 to 68.2) C: 32.3 (20.4) (95% CI 20.2 to 44.3) P=0.141
Two-Minute Walk Test (M), mean (SD) (95% CI) (95% CI) I: 127.5 (36.0) (95% CI 106.2 to 148.8) C: 110.3 (21.1) (95% CI 97.8 to 122.8) P=0.415
Timed Up and Go (s), mean (SD) (95% CI) I: 8.7 (3.1) (95% CI 6.8 to 10.6) C: 10.8 (2.9) (95% CI 9.0 to 12.5) P=0.179
30-Second Chair Stand (counts), mean (SD) (95% CI) I: 7.0 (4.4) (95% CI 5.3 to 10.5) C: 5.4 (3.2) (95% CI 3.5 to 7.3) P=0.130
Inclusion Body Myositis Functional Rating Scale (IBMFRS (10 items 0-40)), mean (SD) (95% CI) I: 32.5 (4.9) (95% CI 29.6 to 35.4) C: 29.7 (4.9) (95% CI 26.8 to 32.6) P=0.018
Inclusion Body Myositis Functional Rating Scale (IBMFRS (5 items 0-20)), mean (SD) (95% CI) I: 14.6 (3.5) (95% CI 12.5 to 16.7) C: 13.2 (3.3) (95% CI 11.3 to 15.1) P=0.353
Knee extensor strength (Nm/kg), mean (SD) (95% CI) I: 0.62 (0.55) (95% CI 0.30 to 0.95) C: 0.53 (0.50) (95% CI 0.24 to 0.83) P=0.168
Myositis Disease Activity Assessment Tool (MDAAT) (0-1), mean (SD) (95% CI) I: 0.10 (0.07) (95% CI 0.06 to 0.14) C: 0.18 (0.12) (95% CI 0.11 to 0.25) P=0.539
Patient Global Activity (0-100), mean (SD) (95% CI) I: 13.5 (25.9) (95% CI -1.8 to 28.9) C: 19.0 (18.5) (95% CI 8.0 to 30.0). P=0.987
Physician Global Activity (0-100), mean (SD) (95% CI) I: 5.9 (1.9) (95% CI 4.1 to 6.3) C: 5.7 (2.7) (95% CI 4.1 to 7.3) P=0.226
Myositis Damage Index (MDI) (0-1), mean (SD) (95% CI) I: 0.19 (0.06) (95% CI 0.16 to 0.23) C: 0.17 (0.07) (95% CI 0.14 to 0.21) P=0.873
Patient global damage (0-100), mean (SD) (95% CI) I: 28.3 (10.7) (95% CI 21.9 to 34.6) C: 46.9 (15.7) (95% CI 37.7 to 56.2) P=0.262
Physician global damage (0-100), mean (SD) (95% CI) I: 33.0 (19.0) (95% CI 21.8 to 44.2) C: 35.8 (9.7) (95% CI 30.1 to 41.5) P=0.436
Health Assessment Questionnaire (HAQ) (0-3), mean (SD) (95% CI) I: 0.89 (0.73) (95% CI 0.46 to 1.32) C: 1.02 (0.79) (95% CI 0.55 to 1.49) P=0.236
Manual Muscle Test (eight muscles) (0-80), mean (SD) (95% CI) I: 71.2 (5.4) (95% CI 68 to 74.4) C: 66.9 (6.1) (95% CI 63.3 to 70.6) P=0.133 |
Authors’ conclusion:
Previous research into the physiological and biomechanical effects of exercise-based rehabilitation in sIBM patients has been based on single-case reports and small-sized (n ≤ 7), non-controlled studies. While most of these investigations demonstrated beneficial effects of training on mechanical muscle function and physical function, the present study failed to detect a direct beneficial effect of 12 weeks of BFR training in the present group of sIBM patients. However, the present regime of BFR training appeared to provide a significant protective effect on lower-limb mechanical muscle function by preventing or delaying the disease-related decline in contractile capacity, which potentially may provide a long-term aid to preserve ambulatory ability in sIBM patients. The present data also suggest that the IBMFRS survey may be a more sensitive tool to evaluate self-reported physical function in sIBM patients than the SF36 health survey. However, this aspect needs to be investigated more thoroughly in future studies. To determine the optimal modality of physical training in sIBM patients, larger (i.e. multicentre) studies with longer intervention periods and employing various types of intervention exercise should be considered, along with more mechanistic studies exploring the range of myocellular and neuromuscular plasticity with exercise in sIBM patients.
|
Quality assessment
|
Study
First author, year |
Appropriate and clearly focused question?1
Yes/no/unclear |
Comprehensive and systematic literature search?2
Yes/no/ unclear |
Description of included and excluded studies?3
Yes/no/unclear |
Description of relevant characteristics of included studies?4
Yes/no/unclear |
Appropriate adjustment for potential confounders in observational studies?5
Yes/no/unclear/not applicable |
Assessment of scientific quality of included studies?6
Yes/no/unclear |
Enough similarities between studies to make combining them reasonable?7
Yes/no/unclear |
Potential risk of publication bias taken into account?8
Yes/no/unclear |
Potential conflicts of interest reported?9
Yes/no/unclear |
|
Voet (2019) |
Yes |
Yes |
Yes |
Yes |
Not applicable |
Yes |
Yes |
Yes |
Yes |
|
Study reference
(first author, publication year) |
Was the allocation sequence adequately generated? a
Definitely yes Probably yes Probably no Definitely no |
Was the allocation adequately concealed?b
Definitely yes Probably yes Probably no Definitely no |
Blinding: Was knowledge of the allocated interventions adequately prevented?c
Were patients blinded?
Were healthcare providers blinded?
Were data collectors blinded?
Were outcome assessors blinded?
Were data analysts blinded? Definitely yes Probably yes Probably no Definitely no |
Was loss to follow-up (missing outcome data) infrequent?d
Definitely yes Probably yes Probably no Definitely no |
Are reports of the study free of selective outcome reporting?e
Definitely yes Probably yes Probably no Definitely no |
Was the study apparently free of other problems that could put it at a risk of bias?f
Definitely yes Probably yes Probably no Definitely no |
Overall risk of bias If applicable/ necessary, per outcome measureg
LOW Some concerns HIGH
|
|
Jørgensen (2018) |
Definitely yes
Reason: Following baseline examinations, patients were allocated to either 12 weeks of BFR training or a non-exercising control group. |
Definitely yes
Reason: using a random 1:1 allocation ratio design |
Patient and healthcare provider blinding was not possible.
Outcome assessors were blinded.
Reason: Clinical examination and outcome tests were performed at Odense University Hospital and at the University of Southern Denmark, using outcome assessors blinded to the group allocation.
|
Definitely yes
Reason: no lost to follow-up in the study. |
Probably yes
Reason: all predefined outcome measures were reported. |
No information
Reason: - |
Low |
|
Wallace (2019) |
Probably yes;
Reason: Participants were randomized to 2 groups.
|
No information;
Reason: - |
Definitely no (patients not blinded);
Reason: We decided to also include an 8-week period between C1 and T2 for group B to maintain blinding of assessors.
It was not possible to blind participants in an exercise trial where aerobic capacity testing was performed using a bicycle ergometer, similar to the training intervention. |
Definitely yes
Reason: no lost to follow-up in the study. |
Probably yes
Reason: all predefined outcome measures were reported. |
No information
Reason: - |
Low |
Table of excluded studies
|
Author and year |
Reason for exclusion |
|
Alemo Munters (2013) |
Duplicate |
|
Alexanderson (2020) |
Does match PICO but included the same studies as the SR of Voet (2019) and was therefore excluded from the analysis. |
|
Baschung Pfister (2015) |
Does match PICO but included the same studies as the SR of Voet (2019) and was therefore excluded from the analysis. |
|
Boehler (2017) |
Wrong outcome measures |
|
Corrado (2020) |
Does match PICO but included the same studies as the SR of Voet (2019) and was therefore excluded from the analysis. |
|
De Oliveira (2018) |
Does match PICO but included the same studies as the SR of Voet (2019) and was therefore excluded from the analysis. |
|
Elnaggar (2021) |
Wrong comparison |
|
Habers (2012) |
Wrong comparison |
|
Habers (2011) |
Does match PICO but included the same studies as the SR of Voet (2019) and was therefore excluded from the analysis. |
|
Jensen (2019) |
Wrong outcome measures |
|
Kant-Smits (2021) |
The study included one RCT that matched our PICO but we already included the RCT in our analysis (Habers, 2016). |
|
McDaid (2017) |
Wrong population |
|
Minniti (2020) |
Wrong population |
|
Alemo Munters (2016) |
Does not match PICO |
|
O Connor (2016) |
Wrong population |
|
Pipitone (2018) |
Wrong study design |
|
Samhan (2020) |
Wrong comparison |
|
Shinjo (2020) |
Wrong study design |
|
Sieczkowska (2021) |
Wrong population |
|
Tiffreau (2017) |
Wrong comparison |
|
Van Thillo (2019) |
Does match PICO but included the same studies as the SR of Voet (2019) and was therefore excluded from the analysis. |
|
Veenhuizen (2019) |
Wrong population |
|
Zhang (2021) |
Does match PICO but included the same studies as the SR of Voet (2019) and was therefore excluded from the analysis. |
Verantwoording
Autorisatiedatum en geldigheid
Laatst beoordeeld : 07-02-2024
Laatst geautoriseerd : 07-02-2024
Geplande herbeoordeling : 01-12-2025
Algemene gegevens
The development of this guideline module was supported by the Knowledge Institute of the Federation of Medical Specialists (www.demedischspecialist.nl/ kennisinstituut) and was financed from the Quality Funds for Medical Specialists (SKMS). The financier has had no influence whatsoever on the content of the guideline module.
Samenstelling werkgroep
A multidisciplinary working group was set up in 2020 for the development of the guideline module, consisting of representatives of all relevant specialisms and patient organisations (see the Composition of the working group) involved in the care of patients with IIM/myositis.
Working group
- Dr. A.J. van der Kooi, neurologist, Amsterdam UMC, location AMC. Nederlandse Vereniging voor Neurologie (chair)
- Dr. U.A. Badrising, neurologist, LUMC. Nederlandse Vereniging voor Neurologie
- Dr. C.G.J. Saris, neurologist, Radboudumc. Nederlandse Vereniging voor Neurologie
- Dr. S. Lassche, neurologist, Zuyderland MC. Nederlandse Vereniging voor Neurologie
- Dr. J. Raaphorst, neurologist, Amsterdam UMC, locatie AMC. Nederlandse Vereniging voor Neurologie
- Dr. J.E. Hoogendijk, neurologist, UMC Utrecht. Nederlandse Vereniging voor Neurologie
- Drs. T.B.G. Olde Dubbelink, neurologist, Rijnstate, Nederlandse Vereniging voor Neurologie
- Dr. I.L. Meek, rheumatologist, Radboudumc. Nederlandse Vereniging voor Reumatologie
- Dr. R.C. Padmos, rheumatologist, Erasmus MC. Nederlandse Vereniging voor Reumatologie
- Prof. dr. E.M.G.J. de Jong, dermatologist, werkzaam in het Radboudumc. Nederlandse Vereniging voor Dermatologie en Venereologie
- Drs. W.R. Veldkamp, dermatologist, Ziekenhuis Gelderse Vallei. Nederlandse Vereniging voor Dermatologie en Venereologie
- Dr. J.M. van den Berg, pediatrician, Amsterdam UMC, locatie AMC. Nederlandse Vereniging voor Kindergeneeskunde
- Dr. M.H.A. Jansen, pediatrician, UMC Utrecht. Nederlandse Vereniging voor Kindergeneeskunde
- Dr. A.C. van Groenestijn, rehabilitation physician, Amsterdam UMC, locatie AMC. Nederlandse Vereniging van Revalidatieartsen
- Dr. B. Küsters, pathologist, Radboudumc. Nederlandse Vereniging voor Pathologie
- Dr. V.A.S.H. Dalm, internist, Erasmus MC. Nederlandse Internisten Vereniging
- Drs. J.R. Miedema, pulmonologist, Erasmus MC. Nederlandse Vereniging van Artsen voor Longziekten en Tuberculose
- I. de Groot, patient representatieve. Spierziekten Nederland
Advisory board
- Prof. dr. E. Aronica, pathologist, Amsterdam UMC, locatie AMC. External expert.
- Prof. dr. D. Hamann, Laboratory specialist medical immunology, UMC Utrecht. External expert.
- Drs. R.N.P.M. Rinkel, ENT physician, Amsterdam UMC, locatie VUmc. Vereniging voor Keel-Neus-Oorheelkunde en Heelkunde van het Hoofd-Halsgebied
- dr. A.S. Amin, cardiologist, werkzaam in werkzaam in het Amsterdam UMC, locatie AMC. Nederlandse Vereniging voor Cardiologie
- dr. A. van Royen-Kerkhof, pediatrician, UMC Utrecht. External expert.
- dr. L.W.J. Baijens, ENT physician, Maastricht UMC+. External expert.
- Em. Prof. Dr. M. de Visser, neurologist, Amsterdam UMC. External expert.
Methodological support
- Drs. T. Lamberts, senior advisor, Knowledge institute of the Federation of Medical Specialists
- Drs. M. Griekspoor, advisor, Knowledge institute of the Federation of Medical Specialists
- Dr. M. M. J. van Rooijen, advisor, Knowledge institute of the Federation of Medical Specialists
Belangenverklaringen
The ‘Code ter voorkoming van oneigenlijke beïnvloeding door belangenverstrengeling’ has been followed. All working group members have declared in writing whether they have had direct financial interests (attribution with a commercial company, personal financial interests, research funding) or indirect interests (personal relationships, reputation management) in the past three years. During the development or revision of a module, changes in interests are communicated to the chairperson. The declaration of interest is reconfirmed during the comment phase.
An overview of the interests of working group members and the opinion on how to deal with any interests can be found in the table below. The signed declarations of interest can be requested from the secretariat of the Knowledge Institute of the Federation of Medical Specialists.
|
Werkgroeplid |
Functie |
Nevenfuncties |
Gemelde belangen |
Ondernomen actie |
|
van der Kooi |
Neuroloog, Amsterdam UMC |
|
Immediate studie (investigator initiated, IVIg behandeling bij therapie naive patienten). --> Financiering via Behring. Studie januari 2019 afgerond |
Geen restricties (middel bij advisory board is geen onderdeel van rcihtlijn) |
|
Miedema |
Longarts, Erasmus MC |
Geen. |
|
Geen restricties |
|
Meek |
Afdelingshoofd a.i. afdeling reumatische ziekten, Radboudumc |
Commissaris kwaliteit bestuur Nederlandse Vereniging voor Reumatologie (onkostenvergoeding) |
Medisch adviseur myositis werkgroep spierziekten Nederland |
Geen restricties |
|
Veldkamp |
AIOS dermatologie Radboudumc Nijmegen |
|
Geen. |
Geen restricties |
|
Padmos |
Reumatoloog, Erasmus MC |
Docent Breederode Hogeschool (afdeling reumatologie EMC wordt hiervoor betaald) |
Geen. |
Geen restricties |
|
Dalm |
Internist-klinisch immunoloog Erasmus MC |
Geen. |
Geen. |
Geen restricties |
|
Olde Dubbelink |
Neuroloog in opleiding Canisius-Wilhelmina Ziekenhuis, Nijmegen |
Promotie onderzoek naar diagnostiek en outcome van het carpaletunnelsyndroom (onbetaald) |
Geen. |
Geen restricties |
|
van Groenestijn |
Revalidatiearts AmsterdamUMC, locatie AMC |
Geen. |
Lokale onderzoeker voor de I'M FINE studie (multicentre, leiding door afdeling Revalidatie Amsterdam UMC, samen met UMC Utrecht, Sint Maartenskliniek, Klimmendaal en Merem. Evaluatie van geïndividualiseerd beweegprogramma o.b.v. combinatie van aerobe training en coaching bij mensen met neuromusculaire aandoeningen, NMA). Activiteiten: screening NMA-patiënten die willen participeren aan deze studie. Subsidie van het Prinses Beatrix Spierfonds. |
Geen restricties |
|
Lassche |
Neuroloog, Zuyderland Medisch Centrum, Heerlen en Sittard-Geleen |
Geen. |
Geen. |
Geen restricties |
|
de Jong |
Dermatoloog, afdelingshoofd Dermatologie Radboudumc Nijmegen |
Geen. |
All funding is not personal but goes to the independent research fund of the department of dermatology of Radboud university medical centre Nijmegen, the Netherlands |
Geen restricties |
|
Hoogendijk |
Neuroloog Universitair Medisch Centrum Utrecht (0,4) Neuroloog Sionsberg, Dokkum (0,6) |
beide onbetaald |
Geen. |
Geen restricties |
|
Badrising |
Neuroloog Leids Universitair Medisch Centrum |
(U.A.Badrising Neuroloog b.v.: hoofdbestuurder; betreft een vrijwel slapende b.v. als overblijfsel van mijn eerdere praktijk in de maatschap neurologie Dirksland, Het van Weel-Bethesda Ziekenhuis) |
Medisch adviseur myositis werkgroep spierziekten Nederland |
Geen restricties |
|
van den Berg |
Kinderarts-reumatoloog/-immunoloog Emma kinderziekenhuis/ Amsterdam UMC |
Geen. |
Geen. |
Geen restricties |
|
de Groot |
Patiënt vertegenwoordiger/ ervaringsdeskundige: voorzitter diagnosewerkgroep myositis bij Spierziekten Nederland in deze commissie patiënt(vertegenwoordiger) |
|
Geen |
Geen restricties |
|
Küsters |
Patholoog, Radboud UMC |
Geen. |
Geen. |
Geen restricties |
|
Saris |
Neuroloog/ klinisch neurofysioloog, Radboudumc |
Geen. |
Geen. |
Geen restricties |
|
Raaphorst |
Neuroloog, Amsterdam UMC |
Geen. |
|
Restricties m.b.t. opstellen aanbevelingen IvIg behandeling. |
|
Jansen |
Kinderarts-immunoloog-reumatoloog, WKZ UMC Utrecht |
Docent bij Mijs-instituut (betaald) |
Onderzoek biomakers in juveniele dermatomyositis. Geen belang bij uitkomst richtlijn. |
Geen restricties |
Inbreng patiëntenperspectief
Attention was paid to the patient's perspective by offering the Vereniging Spierziekten Nederland to take part in the working group. Vereniging Spierziekten Nederland has made use of this offer, the Dutch Artritis Society has waived it. In addition, an invitational conference was held to which the Vereniging Spierziekten Nederland, the Dutch Artritis Society nd Patiëntenfederatie Nederland were invited and the patient's perspective was discussed. The report of this meeting was discussed in the working group. The input obtained was included in the formulation of the clinical questions, the choice of outcome measures and the considerations. The draft guideline was also submitted for comment to the Vereniging Spierziekten Nederland, the Dutch Artritis Society and Patiëntenfederatie Nederland, and any comments submitted were reviewed and processed.
Qualitative estimate of possible financial consequences in the context of the Wkkgz
In accordance with the Healthcare Quality, Complaints and Disputes Act (Wet Kwaliteit, klachten en geschillen Zorg, Wkkgz), a qualitative estimate has been made for the guideline as to whether the recommendations may lead to substantial financial consequences. In conducting this assessment, guideline modules were tested in various domains (see the flowchart on the Guideline Database).
The qualitative estimate shows that there are probably no substantial financial consequences, see table below.
|
Module |
Estimate |
Explanation |
|
Module diagnostische waarde ziekteverschijnselen |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Optimale strategie aanvullende diagnostiek myositis |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Autoantibody testing in myositis |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Screening op maligniteiten |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Screening op comorbiditeiten |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Immunosuppressie en -modulatie bij IBM |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Treatment with Physical training |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Treatment of dysphagia in myositis |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Treatment of dysphagia in IBM |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Topical therapy |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Treatment of calcinosis |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
|
Module Organization of care |
No substantial financial consequences |
Outcome 1 No financial consequences. The recommendations are not widely applicable (<5,000 patients) and are therefore not expected to have any substantial financial consequences on collective expenditures. |
Werkwijze
Methods
AGREE
This guideline module has been drawn up in accordance with the requirements stated in the Medisch Specialistische Richtlijnen 2.0 report of the Advisory Committee on Guidelines of the Quality Council. This report is based on the AGREE II instrument (Appraisal of Guidelines for Research & Evaluation II; Brouwers, 2010).
Clinical questions
During the preparatory phase, the working group inventoried the bottlenecks in the care of patients with IIM. Bottlenecks were also put forward by the parties involved via an invitational conference. A report of this is included under related products.
Based on the results of the bottleneck analysis, the working group drew up and finalized draft basic questions.
Outcome measures
After formulating the search question associated with the clinical question, the working group inventoried which outcome measures are relevant to the patient, looking at both desired and undesired effects. A maximum of eight outcome measures were used. The working group rated these outcome measures according to their relative importance in decision-making regarding recommendations, as critical (critical to decision-making), important (but not critical), and unimportant. The working group also defined at least for the crucial outcome measures which differences they considered clinically (patient) relevant.
Methods used in the literature analyses
A detailed description of the literature search and selection strategy and the assessment of the risk-of-bias of the individual studies can be found under 'Search and selection' under Substantiation. The assessment of the strength of the scientific evidence is explained below.
Assessment of the level of scientific evidence
The strength of the scientific evidence was determined according to the GRADE method. GRADE stands for Grading Recommendations Assessment, Development and Evaluation (see http://www.gradeworkinggroup.org/). The basic principles of the GRADE methodology are: naming and prioritizing the clinically (patient) relevant outcome measures, a systematic review per outcome measure, and an assessment of the strength of evidence per outcome measure based on the eight GRADE domains (downgrading domains: risk of bias, inconsistency, indirectness, imprecision, and publication bias; domains for upgrading: dose-effect relationship, large effect, and residual plausible confounding).
GRADE distinguishes four grades for the quality of scientific evidence: high, fair, low and very low. These degrees refer to the degree of certainty that exists about the literature conclusion, in particular the degree of certainty that the literature conclusion adequately supports the recommendation (Schünemann, 2013; Hultcrantz, 2017).
|
Definitie |
|
|
High |
|
|
Moderate |
|
|
Low |
|
|
Very low |
|
When assessing (grading) the strength of the scientific evidence in guidelines according to the GRADE methodology, limits for clinical decision-making play an important role (Hultcrantz, 2017). These are the limits that, if exceeded, would lead to an adjustment of the recommendation. To set limits for clinical decision-making, all relevant outcome measures and considerations should be considered. The boundaries for clinical decision-making are therefore not directly comparable with the minimal clinically important difference (MCID). Particularly in situations where an intervention has no significant drawbacks and the costs are relatively low, the threshold for clinical decision-making regarding the effectiveness of the intervention may lie at a lower value (closer to zero effect) than the MCID (Hultcrantz, 2017).
Considerations
In addition to (the quality of) the scientific evidence, other aspects are also important in arriving at a recommendation and are taken into account, such as additional arguments from, for example, biomechanics or physiology, values and preferences of patients, costs (resource requirements), acceptability, feasibility and implementation. These aspects are systematically listed and assessed (weighted) under the heading 'Considerations' and may be (partly) based on expert opinion. A structured format based on the evidence-to-decision framework of the international GRADE Working Group was used (Alonso-Coello, 2016a; Alonso-Coello 2016b). This evidence-to-decision framework is an integral part of the GRADE methodology.
Formulation of conclusions
The recommendations answer the clinical question and are based on the available scientific evidence, the most important considerations, and a weighting of the favorable and unfavorable effects of the relevant interventions. The strength of the scientific evidence and the weight assigned to the considerations by the working group together determine the strength of the recommendation. In accordance with the GRADE method, a low evidential value of conclusions in the systematic literature analysis does not preclude a strong recommendation a priori, and weak recommendations are also possible with a high evidential value (Agoritsas, 2017; Neumann, 2016). The strength of the recommendation is always determined by weighing all relevant arguments together. The working group has included with each recommendation how they arrived at the direction and strength of the recommendation.
The GRADE methodology distinguishes between strong and weak (or conditional) recommendations. The strength of a recommendation refers to the degree of certainty that the benefits of the intervention outweigh the harms (or vice versa) across the spectrum of patients targeted by the recommendation. The strength of a recommendation has clear implications for patients, practitioners and policy makers (see table below). A recommendation is not a dictate, even a strong recommendation based on high quality evidence (GRADE grading HIGH) will not always apply, under all possible circumstances and for each individual patient.
|
Implications of strong and weak recommendations for guideline users |
||
|
|
||
|
|
Strong recommendation |
Weak recommendations |
|
For patients |
Most patients would choose the recommended intervention or approach and only a small number would not. |
A significant proportion of patients would choose the recommended intervention or approach, but many patients would not. |
|
For practitioners |
Most patients should receive the recommended intervention or approach. |
There are several suitable interventions or approaches. The patient should be supported in choosing the intervention or approach that best reflects his or her values and preferences. |
|
For policy makers |
The recommended intervention or approach can be seen as standard policy. |
Policy-making requires extensive discussion involving many stakeholders. There is a greater likelihood of local policy differences. |
Organization of care
In the bottleneck analysis and in the development of the guideline module, explicit attention was paid to the organization of care: all aspects that are preconditions for providing care (such as coordination, communication, (financial) resources, manpower and infrastructure). Preconditions that are relevant for answering this specific initial question are mentioned in the considerations. More general, overarching or additional aspects of the organization of care are dealt with in the module Organization of care.
Commentary and authtorisation phase
The draft guideline module was submitted to the involved (scientific) associations and (patient) organizations for comment. The comments were collected and discussed with the working group. In response to the comments, the draft guideline module was modified and finalized by the working group. The final guideline module was submitted to the participating (scientific) associations and (patient) organizations for authorization and authorized or approved by them.
References
Agoritsas T, Merglen A, Heen AF, Kristiansen A, Neumann I, Brito JP, Brignardello-Petersen R, Alexander PE, Rind DM, Vandvik PO, Guyatt GH. UpToDate adherence to GRADE criteria for strong recommendations: an analytical survey. BMJ Open. 2017 Nov 16;7(11):e018593. doi: 10.1136/bmjopen-2017-018593. PubMed PMID: 29150475; PubMed Central PMCID: PMC5701989.
Alonso-Coello P, Schünemann HJ, Moberg J, Brignardello-Petersen R, Akl EA, Davoli M, Treweek S, Mustafa RA, Rada G, Rosenbaum S, Morelli A, Guyatt GH, Oxman AD; GRADE Working Group. GRADE Evidence to Decision (EtD) frameworks: a systematic and transparent approach to making well informed healthcare choices. 1: Introduction. BMJ. 2016 Jun 28;353:i2016. doi: 10.1136/bmj.i2016. PubMed PMID: 27353417.
Alonso-Coello P, Oxman AD, Moberg J, Brignardello-Petersen R, Akl EA, Davoli M, Treweek S, Mustafa RA, Vandvik PO, Meerpohl J, Guyatt GH, Schünemann HJ; GRADE Working Group. GRADE Evidence to Decision (EtD) frameworks: a systematic and transparent approach to making well informed healthcare choices. 2: Clinical practice guidelines. BMJ. 2016 Jun 30;353:i2089. doi: 10.1136/bmj.i2089. PubMed PMID: 27365494.
Brouwers MC, Kho ME, Browman GP, Burgers JS, Cluzeau F, Feder G, Fervers B, Graham ID, Grimshaw J, Hanna SE, Littlejohns P, Makarski J, Zitzelsberger L; AGREE Next Steps Consortium. AGREE II: advancing guideline development, reporting and evaluation in health care. CMAJ. 2010 Dec 14;182(18):E839-42. doi: 10.1503/cmaj.090449. Epub 2010 Jul 5. Review. PubMed PMID: 20603348; PubMed Central PMCID: PMC3001530.
Hultcrantz M, Rind D, Akl EA, Treweek S, Mustafa RA, Iorio A, Alper BS, Meerpohl JJ, Murad MH, Ansari MT, Katikireddi SV, Östlund P, Tranæus S, Christensen R, Gartlehner G, Brozek J, Izcovich A, Schünemann H, Guyatt G. The GRADE Working Group clarifies the construct of certainty of evidence. J Clin Epidemiol. 2017 Jul;87:4-13. doi: 10.1016/j.jclinepi.2017.05.006. Epub 2017 May 18. PubMed PMID: 28529184; PubMed Central PMCID: PMC6542664.
Medisch Specialistische Richtlijnen 2.0 (2012). Adviescommissie Richtlijnen van de Raad Kwaliteit. http://richtlijnendatabase.nl/over_deze_site/over_richtlijnontwikkeling.html
Neumann I, Santesso N, Akl EA, Rind DM, Vandvik PO, Alonso-Coello P, Agoritsas T, Mustafa RA, Alexander PE, Schünemann H, Guyatt GH. A guide for health professionals to interpret and use recommendations in guidelines developed with the GRADE approach. J Clin Epidemiol. 2016 Apr;72:45-55. doi: 10.1016/j.jclinepi.2015.11.017. Epub 2016 Jan 6. Review. PubMed PMID: 26772609.
Schünemann H, Brożek J, Guyatt G, . GRADE handbook for grading quality of evidence and strength of recommendations. Updated October 2013. The GRADE Working Group, 2013. Available from http://gdt.guidelinedevelopment.org/central_prod/_design/client/handbook/handbook.html.
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