Optimal strategy for additional diagnostics in myositis
Uitgangsvraag
Which diagnostic tests should be used for diagnosing idiopathic inflammatory myopathy (IIM)?
Welke plaats heeft aanvullende diagnostiek bij het vaststellen van de diagnose IIM?
Aanbeveling
Aanbeveling IIM (exclusief IBM en JDM)
Stel de diagnose myositis op basis van een combinatie van:
- Anamnese en lichamelijk onderzoek (incl. neurologisch onderzoek en huidonderzoek);
- Laboratoriumonderzoek (waarbij een normaal CK de diagnose myositis niet uitsluit);
- En histologie* met beoordeling van de morfologie (biopt afgenomen voorafgaand aan of binnen 1 week na start immunomodulerende behandeling/immunosuppressiva, bij voorkeur in een centrum met expertise).
Aanvullende diagnostiek bij de verdenking op myositis kan worden ingezet voor verschillende doeleinden:
- Verricht een MRI spieren voor de plaatsbepaling van het spierbiopt. Verricht alleen een MRI in een centrum waar ervaring is met de verrichting en interpretatie van deze beeldvorming.
- Verricht niet standaard een EMG. Dit heeft onvoldoende diagnostische waarde, maar kan wel worden ingezet om onderscheid te maken binnen een brede differentiaaldiagnose.
- Overweeg enkel spierechografie te verrichten (beoordeling toegenomen echo-intensiteit en/of het voorkomen van spieroedeem en fasciitis, ter vervanging van een MRI) in een centrum met uitgebreide ervaring.
- Overweeg enkel een CT te verrichten indien er contra-indicaties voor MRI bestaan en met het doel vervetting en/of atrofie in beeld te brengen voorafgaand aan een spierbiopt.
Indien beeldvorming niet mogelijk is, neem dan een spierbiopt af bij voorkeur in een klinisch aangedane spier.
* Overweeg af te zien van een spierbiopt:
- Bij patiënten bekend met een Connective Tissue Disease, evidente spierzwakte en een verhoogd CK
- Bij een klassieke presentatie van dermatomyositis
- Bij een harde indicatie voor therapeutische antistolling met een hoog risico op complicaties bij tijdelijk couperen (bijv. mechanische hartklep)
- Indien biopteerbare spieren zeer atrofisch of vervet zijn.
Aanbevelingen IBM
Als aanpassingen op de aanbevelingen voor IIM geldt voor IBM:
- Stel de diagnose IBM op basis van het klinisch beeld en de histologie
- Verricht bij voorkeur beeldvorming voorafgaand aan het spierbiopt
- Overweeg aanvullend onderzoek naar subklinische betrokkenheid van de diepe vingerflexoren middels echo, MRI of naaldEMG, ter ondersteuning van de diagnose.
Aanbevelingen JDM
Stel de diagnose JDM in een centrum met voldoende expertise op basis van de klinische presentatie:
- Objectieve symmetrische, proximale (meer dan distale) spierzwakte die chronisch en meestal progressief is;
- Cutane symptomen (heliotrope uitslag, Gottronse papels en/of het Gottrons sign)
De diagnose kan verder worden onderbouwd met:
- De serologie (CK, LDH, ASAT, ALAT, aldolase; waarbij een normaal CK de diagnose myositis niet uitsluit)
- Overweeg in een kleine minderheid van de patienten een spierbiopt
- Verricht geen EMG
Aanvullende diagnostiek ter ondersteuning van de diagnose kan overwogen worden:
- Nagelriem videocapillairoscopie
- Beeldvorming: MRI of echo
- Interferonsignatuur in bloed
Behandel JDM in een academisch centrum onder begeleiding van een kinderreumatoloog.
Overwegingen
Considerations – from evidence to recommendation
Pros and cons of the intervention and the quality of the evidence
IIM
The current diagnosis of IIM is based on (1) the patient’s symptoms and signs (including thorough clinical examination), (2) laboratory values, and (3) evidence from histological samples.
Based on the evidence from the included literature (low or very low grade), additional use of diagnostic modalities does not seem to contribute to establishing the diagnose, yet can be useful for other aspects of the diagnostic trajectory.
MRI
The low levels of evidence of the literature suggest that neither EMG measurements, nor MRI, nor ultrasound can replace muscle biopsy for diagnosing inflammatory myopathies. However, this low level of evidence (GRADE) means that new (future) research can probably alter the conclusion. Concerning MRI, this seems to have sufficient sensitivity, specificity, and positive predictive value when performed as triage test for muscle biopsy. However, this conclusion is based on only one study with a low number of included patients. In this study, the degree of involved muscle with high-signal intensity on T2-weighted images was assessed, and the presence of fatty infiltration in muscle on T1-weighted images. This assessment seems (partly) similar to the edema and fatty replacement measurements of the other studies that assessed MRI, and which showed much lower diagnostic accuracy estimates. Possibly MRI could be adopted as triage test for a muscle biopsy, yet the level of evidence is low (meaning new research could probably alter the conclusion).
MRI is nonetheless useful for the determination of the location for biopsy (irrespective of its potential use in triaging patients for biopsy). An MRI prior to muscle biopsy can prevent taking a sample from a normal muscle or muscle with too much fatty infiltration, and increases the chance of finding (pathological) alterations in the muscle tissue, which could contribute to a diagnosis. Additionally, an MRI (T2-weighted) can provide valuable information about the pattern and extent of muscle involvement, which can for example aid in differentiating IBM from other types of myositis and help monitor treatment effects. It can assist in tracking the response to treatment, by showing improvements or deteriorations in muscle tissue (Malartre, 2021).
EMG
For EMG, the current conclusion is based on different types of EMG measurement, and a wide dispersion of estimates of accuracy is found. The low level of evidence (GRADE) means that new (future) research could probably alter the conclusion. The best values for sensitivity were found for the measurement of short motor unit potentials, but this has been researched in only one study. These findings are known to suggest a myopathy, and EMG could therefore discriminate between neurogenic causes and IIM. A disadvantage of EMG measurement is that it is invasive and considered painful by some.
Ultrasound
Muscle perfusion parameters (blood volume, flow, and velocity) on contrast enhanced ultrasound examinations did not result in additional diagnostic information to diagnose IIM. The level of evidence is very low, mostly because the basis for the evidence is a single study with a low number of patients (and an extremely high suspicion of IIM), and with multiple limitations in study design and execution. In current practice, few Dutch centres use ultrasonography in the diagnostic trajectory of IIM, using Z-values for the quantification of echo-intensity (which are other than muscle perfusion parameters as described above). Interpretation of these values is difficult, therefore extensive experience is required. Nonetheless, in some cases it could be used instead MRI for the determination of the muscle biopsy location.
CT
A CT scan cannot be used to diagnose IIM as only fatty replacement and atrophy of muscles is visible, and it has the disadvantage of additional radiation exposure.
Muscle biopsy
Muscle biopsy is usually considered the gold standard for diagnosing IIM. In case of reasonable suspicion of IIM, muscle biopsy is performed to demonstrate the inflammatory alteration in the muscle or to rule out other differential diagnoses. Subtyping of inflammatory reaction (Allenbach, 2017) may be easier if larger pieces of muscle tissue are obtained (open biopsy, conchotome biopsy); this can be difficult in needle biopsy material. The advantage of needle biopsy is the quick process of obtaining the biopsy sample without the need of a surgeon and a smaller scar. The scar is also much smaller in a conchotome biopsy. Local setting and experience will influence the preferred method of muscle biopsy: open surgical, conchotome or needle biopsy. The recommendations of the EURO-NMD pathology working group can be followed for further pathological/histological work-up (Udd, 2019).
Although one of the central pillars of the IIM diagnosis, a muscle biopsy also has its limitations. Muscle histology does not guarantee a diagnosis and could be inconclusive (or negative) due to sampling error, too much fibrosis or fatty replacement in the muscle, or because treatment has already been started and has influenced the result. A muscle biopsy could also show only a few – not all – of the abnormalities required for diagnosis. Therefore, muscle histology can be considered to be omitted if the result is not likely to alter the diagnosis. This could be the case in the presence of typical skin lesions or a combination of specific antibodies and muscle weakness.
It is important to weigh the potential benefits and risks of a muscle biopsy. Obtaining histological samples can pose risks for patients, for example allergic reactions to the anaesthetics, pain, wound infection, bleeding, and scarring (Ross, 2023). The decision to perform a muscle biopsy should also be re-evaluated in cases where stopping anticoagulant therapy may pose significant risks to the patient’s health (e.g. in patients with a mechanical heart valve), or when a muscle biopsy is unlikely to be successful due to extreme muscle atrophy. In such cases, it may be more appropriate to skip the procedure and consider alternative methods to support a diagnosis.
(J)DM
Similar to adults, the diagnosis IIM in children (JDM) is primarily based on the clinical signs and symptoms and laboratory results (i.e.: elevated CK, LDH, ASAT, ALAT, aldolase). Paediatric patients are classified as JDM when they have objective, symmetrical, proximal (more than distal) muscle weakness that is chronic and usually progressive, and have additional heliotrope rash, Gottron’s papules and/or Gottron’s sign, and nailfold capillary changes.
Additional investigations can support the diagnosis but are not required to demonstrate or rule out JDM. Typical signs of myositis on MRI or muscle ultrasonography support the diagnosis, as does the presence of myositis-specific autoantibodies, myositis associated antibodies and increased levels of certain biomarkers (such as Galectin-9, CXCL10 or interferon-signature) (Wienke, 2019), which can also be used for the monitoring of disease activity. However, their presence is not a criterium for JDM.
EMG is not routinely performed, but can be useful in differentiating myopathy from neuropathy. If available, nailfold capillaroscopy is advised but not obligatory. Presence of a “scleroderma pattern” may help to support the diagnosis of JDM, and this is a non-invasive test (Shenavandeh, 2022).
With regard to histopathology, in contrast to adults, a muscle biopsy should be reserved for children in whom doubt exists about the diagnosis, e.g. in those with an incomplete phenotype or unexplained symptoms, such as the lack of typical cutaneous manifestations (in which there is no preference to either an open biopsy or a needle biopsy). A skin biopsy can be considered, however findings are usually not specific.
Confirmation of the diagnosis and treatment of JDM should take place in an academic centre under the supervision of a pediatric rheumatologist.
IBM
To diagnose IBM, a meticulous clinical assessment is key. The physical examination should include the testing of deep and superficial finger flexor strength (Greenberg, 2016). Similar to the diagnostic trajectory of IIM, EMG can support the myogenic origin of IBM, yet cannot replace muscle biopsy. Myography of involved muscles can show a combination of neurogenic and myopathic abnormalities at first sight (Greenberg, 2016), but becomes more myogenic after motor unit number estimation. Also ultrasonography and MRI can contribute to the diagnosis of IBM, as these diagnostic modalities can help differentiate between IBM and its mimics (Abdelnaby, 2022; Leeuwenberg, 2020; Noto, 2014; Cox, 2011; Tasca, 2015). Only when MRI is contraindicated, a CT-scan can be performed to assess the pattern of fatty replacement in muscles, which has no diagnostic value in itself but can support IBM diagnosis or help in locating an appropriate spot for muscle biopsy (Futura, 2020).
The diagnostic value of auto-antibodies (including anti-cN1A) is covered in module Auto-antibody testing in myositis.
Values and preferences of patients
Patients' values and preferences about diagnostic and treatment options for IIM should be considered in shared decision-making with physicians. Risks and benefits of different diagnostic modalities (MRI, biopsy, EMG) should be explained and discussed. Invasive tests such as biopsy and EMG can be painful and biopsy poses temporary limitations in activities of daily living (e.g., no swimming for one week), but it may well offer a definitive diagnosis. MRI might aid in finding the right location for muscle biopsy (decreasing the chance of a repeat muscle biopsy), yet may not be possible for some patients (e.g., claustrophobia, certain pacemakers). The diagnostic process may be lengthy and therefore possibly delay the start of treatment, but a definitive diagnosis provides certainty. A definitive diagnosis is more difficult to establish later, because interpretation of tests can be hampered once treatment has started, or a biopsy may be difficult to take due to the fatty replacement in muscles. In addition, a definitive diagnosis could be important for access to clinical trials and new treatments.
Costs
Patients with IIM incur significant costs to society due to the chronic and multi-faceted nature of the disease. Treatment requires ongoing and potentially expensive medication and rehabilitation, frequent medical visits, and screening for related conditions, such as malignancies or osteoporosis and adaptations to living, care and transport conditions. The cost of a diagnostic trajectory is relatively minimal compared to the overall costs of care, but a biopsy is more expensive (when also considering the anaesthesia/local anaesthetic, histopathologic staining and the report) than MRI or EMG (Ahmed, 2022). An MRI prior to biopsy may also reduce the need for a second biopsy.
Acceptability, feasibility and implementation
Current recommendations are in line with current practice. However, there are some barriers in the current diagnostic trajectory that impact feasibility or acceptability:
- Long wait times for MRI can cause frustration and (unnecessary) delay in IIM diagnosis and treatment. For IBM, the diagnostic trajectory is allowed to take longer, because of the chronic nature of the condition without a (current) effective therapy.
- MRI interpretation. An MRI should be performed in a centre with a radiologist experienced in the interpretation of muscle abnormalities.
- Biopsy experience. Biopsy material should be processed and interpreted in a pathology laboratory with significant experience in the processing and storage of muscle tissue (isopentane frozen material), with a pathologist who is experienced in interpreting muscle pathology. This is preferably an academic hospital, as considerable risk exists that the sample is processed or treated in the wrong way (e.g., tissue mounting in paraffin instead of using frozen sections), requiring a repeat muscle biopsy; or that unfamiliarity with muscle pathology in general hinders diagnosis.
Recommendations
1. Recommendations IIM
De diagnose IIM (“myositis”) is gebaseerd op een gedegen anamnese, klinische verschijnselen, het laboratoriumonderzoek en het spierbiopt. Aanvullend kunnen diagnostische modaliteiten worden ingezet ter ondersteuning van het nemen van het spierbiopt of ter differentiatie van neurogene aandoeningen of ander myopathieën. Een biopt is niet altijd van meerwaarde en brengt risico’s met zich mee, en kan achterwege worden gelaten indien dit een onacceptabel risico voor de patiënt vormt.
2. Recommendations IBM
De diagnose inclusion body myositis (IBM) is gebaseerd op een gedegen anamnese, klinische verschijnselen en het spierbiopt. Aanvullend kunnen diagnostische modaliteiten worden ingezet ter ondersteuning van het nemen van het spierbiopt of ter differentiatie van neurogene aandoeningen of mimics. Een spierbiopt heeft inherente tekortkomingen en kan in uitzonderlijke gevallen achterwege worden gelaten.
Onderbouwing
Achtergrond
To diagnose idiopathic inflammatory myopathy (IIM, “myositis”) a muscle biopsy – a relatively invasive procedure – is considered the gold standard, except for JDM. However, according to certain guidelines (NVN & ISNO, 2005) a muscle biopsy is not always required for the diagnosis of certain subtypes of IIM. The diagnosis dermatomyositis (DM) – for example – could be made based on suggestive skin lesions for DM in combination with skin biopsy changes alone. Apart from specific skin lesions for DM, serum Creatine Kinase, (CK), electromyography (EMG), muscle imaging (MRI or ultrasound (US)) could contribute to the diagnostic process of IIM. However, their diagnostic value remains unclear for patients with suspected IIM; yet in case of high test accuracy, these tests could prevent the need for additional muscle biopsy.
Conclusies
|
Low GRADE |
EMG The evidence suggests that for (specific measurements -including combinations of) electromyography or muscle fiber conduction velocity, the sensitivity varies from 0.02 to 0.97, the specificity from 0.33 to 0.93, and the positive predictive value from 0.08 to 0.71 in patients suspected of (inflammatory) myopathies. Therefore no conclusion can be drawn on its clinical value or applicability.
Source: Blijham 2004, Lehmann Urban 2021, Sener 2019 |
|
Low GRADE |
MRI – in general, as add-on test, or as triage test There is limited evidence that magnetic resonance imaging (MRI) might have sufficient sensitivity and positive predictive value (varying from 0.81 to 0.94 and 0.89 to 1.00, respectively). As for specificity, the limited evidence only supports the use of MRI as triage test. No conclusion can be drawn as to which of these uses of MRI would lead to the best clinical outcomes.
Source: Van de Vlekkert 2015 |
|
Low GRADE |
MRI – oedema The evidence suggests that for measurement of oedema on magnetic resonance imaging, the sensitivity varies from 0.40 to 1.00, the specificity from 0.50 to 0.88, and the positive predictive value from 0.26 to 0.77 in patients suspected of (a specific) myopathy.
Source: Lehmann Urban 2021, Weber 2006 |
|
Low GRADE |
MRI – atrophy, fatty replacement, and contrast uptake The evidence suggests that for measurement of atrophy, fatty replacement or contrast uptake on magnetic resonance imaging, the estimates of diagnostic accuracy are low: the sensitivity varies from 0.24 to 0.61, the specificity from 0.30 to 0.70, and the positive predictive value from 0.21 to 0.24 in patients suspected of myopathy.
Source: Lehmann Urban 2021 |
|
Very low GRADE |
Ultrasound The evidence is uncertain about the sensitivity, specificity, and positive predictive value of muscle perfusion measured on contrast-enhanced ultrasound, for patients with suspected polymyositis or dermatomyositis.
Source: Weber 2006 |
Samenvatting literatuur
Description of studies
The following studies investigated diagnostic modalities for IIM:
Blijham (2004) studied the value of muscle fiber conduction velocity (MFCV) in the diagnostic work-up of patients presenting with clinical signs and symptoms suggestive of inflammatory myopathy. To this end, all such patients who presented at the institution in 1999 were included, and compared to healthy controls. Exclusion criteria were not reported. Included patients underwent needle electromyography (EMG), MFCV measurement and subsequently a muscle biopsy. The EMG was regarded as typical for IIM if motor unit action potentials (MUAP) showed polyphasia and marked reduction in duration (<8 ms) and height (<1000 microV) in >2 muscles, and spontaneous activity in ≥2 muscles. In the muscle biopsy, muscle changes (atrophic fibers, sarcolemmal expression of major histocompatibility-complex class I antigens, mononuclear cellular infiltrates, perifascicular atrophy of fibers, other changes) were assessed to diagnose IIM. The thresholds for MFCV were calculated based on obtained data. The diagnostic value of MFCV, EMG, and both examinations combined, were determined.
Lehmann Urban (2021) analysed the use of qualitative magnetic resonance imaging (MRI), EMG and muscle biopsy in different cases of clinically suspected myopathy (both inflammatory and non-inflammatory). A total of 191 patients were included with unclear inclusion criteria. Magnetic Resonance Imaging (MRI) of upper or lower extremities or paravertebral muscles was performed in these patients, along with EMG, which occurred prior to muscle biopsy and genetic testing. The MRI was assessed according to previously published grading scores for muscle size (score 0 to 3), muscle fatty replacement (score 0 to 4), muscle edema (score 0 to 2) and extent of contrast enhancement (score 0 to 3). The results of EMG were classified into myopathic, neurogenic, combined or normal, based on previously described normal values for motor unit action potentials. The reference testing through muscle biopsy was categorized as inconspicuous, unspecific myopathic, degenerative myopathic, inflammatory myopathic or neurogenic, based on previously described guidelines.
Sener (2019) assessed whether specific needle EMG findings could be correlated to pathologic findings in muscle biopsy, and therefore to specific types of myopathy, including IIM. Consecutive adult patients who had been referred for muscle biopsy were retrospectively reviewed. In order to be included, patients needed to have undergone an EMG prior to muscle biopsy, which was performed in the contralateral muscle of the needle EMG. EMG interpretation was based on the presence of fibrillation potentials (fibs), myotonic discharges, and short duration muscle unit potentials (MUP). Muscle biopsy reports were reviewed for inflammation, necrosis, fiber splitting and vacuolar changes.
In the single arm intervention study of Van de Vlekkert (2015), the diagnostic accuracy of MRI as a triage test before muscle biopsy, and as add-on test was assessed, in consecutive patients with suspected subacute-onset IIM. The extensive exclusion criteria are summarised in the evidence table. Patients underwent an MRI, which was judged by a radiologist as “in accordance with an inflammatory myopathy” or as “normal or showing non-specific changes”, based on semi-quantitative scoring of the degree of muscle involvement of high signal intensity on T2-weighted images (score 0 to 2), and based on occurrence of fatty infiltration in skeletal muscle (T1-weighted). In addition to MRI, patients underwent muscle biopsy as control test, which was labelled by a neurologist unaware of MRI findings and clinical information as (1) polymyositis (PM), dermatomyositis (DM), non-specific myositis (NSM), or necrotizing auto-immune myopathy (NAM; for this guideline labelled as IMNM), as (2) IBM or a different neuromuscular disease, or as (3) normal/ non-specific. Final diagnosis was based on the European Neuromuscular Centre (ENMC) criteria and a positive response to corticosteroids during follow-up, which was defined as showing improvement of symptoms and muscle strength. The results from MRI, muscle pathology and final diagnosis were compared.
Weber (2006) aimed to evaluate contrast-enhanced ultrasound (CEUS) in patients suspected of PM or DM. A clinical suspicion for PM or DM was present after extensive neurological and dermatological examinations (symptoms of limb weakness, raised serum CK, myopathic findings on EMG, and/or characteristic skin affections). The 35 patients included underwent muscle MRI, muscle biopsy, and CEUS. MRI was assessed for muscle oedema as a sign of myositis. The muscle biopsies were assessed by a neuropathologist for several aspects (named in the evidence table), based on which a dichotomous decision was made whether criteria for myositis were met. CEUS was performed after MRI yet prior to muscle biopsy, and a range of possible cut-offs for different muscle perfusion parameters were calculated through receiver-operating characteristic (ROC) analysis. The results from MRI; the volume, flow and velocity on CEUS; and the muscle biopsy results were compared.
No studies of sufficient evidence (from the performed search) were included in the literature analysis for diagnostic modalities for JDM and IBM.
Results
The following results apply for the diagnosis of IIM (including patients with suspicion of inflammatory myopathy).
Needle Electromyography (EMG) and muscle fiber conduction velocity measurement (MFCV)
Blijham (2004) included 27 patients and 24 healthy controls, in whom EMG and MFCV measurement was conducted. How healthy controls were selected is unclear. Two patients were excluded due to insufficient muscle fiber potentials to calculate MFCV. Thirteen patients were diagnosed with IIM, of which seven with PM, three with DM, and three with inclusion body myositis (IBM). Of the other patients, six had a non-inflammatory myopathy, and in six no neuromuscular disease was diagnosed. Cut-off values for abnormal MFCV were set on <2.8 meter/second or a fastest-to-slowest fiber ratio of >2.2.
Lehmann Urban (2021) included 191 included patients, who were referred to the neurology department between 2014 and 2017, and received diagnostic work-up through serum creatine kinase (CK) measurement, muscle MRI, EMG and muscle biopsy. A total of 51 patients were diagnosed with an inflammatory myopathy. EMG findings of myopathic changes were correlated to the final diagnosis.
Sener (2019) reviewed 224 patients, of whom six did not have an EMG prior to the biopsy, leaving 218 patients for analysis. A total of 62 patients was diagnosed with IIM. The presence of different specific EMG findings (fibs, short duration motor unit potentials, a combination of both, or myotonic discharges) were related to final diagnosis.
The sensitivity, specificity, and positive predictive value of EMG and/or MFCV are depicted in table 1.
|
Study |
Test |
Sensitivity (95%CI) |
Specificity (95%CI) |
Positive predictive value (95%CI) |
|
Blijham (2004) |
Needle EMG* |
0.85 (0.55 to 0.98) |
0.33 (0.10 to 0.65) |
0.58 (0.36 to 0.80) |
|
MFCV |
0.77 (0.46 to 0.95) |
0.42 (0.15 to 0.72) |
0.59 (0.35 to 0.82) |
|
|
EMG + MFCV |
0.77 (0.46 to 0.95) |
0.67 (0.35 to 0.90) |
0.71 (0.48 to 0.95) |
|
|
Lehmann Urban (2021) |
EMG – myopathic changes |
0.57 (0.42 to 0.71) |
0.56 (0.48 to 0.65) |
0.32 (0.23 to 0.42) |
|
Sener (2019) |
EMG – fibs |
0.73 (0.60 to 0.83) |
0.63 (0.55 to 0.70) |
0.44 (0.34 to 0.53) |
|
EMG – short MUP |
0.97 (0.89 to 1.00) |
0.40 (0.33 to 0.49) |
0.39 (0.32 to 0.47) |
|
|
EMG – fibs + short MUP |
0.71 (0.58 to 0.82) |
0.70 (0.62 to 0.77) |
0.48 (0.38 to 0.59) |
|
|
EMG – myotonic discharge |
0.02 (0.00 to 0.09) |
0.93 (0.88 to 0.96) |
0.08 (0.00 to 0.24) |
Table 1. diagnostic measures for EMG and/or MFCV for patients suspected of IIM. EMG: electromyography, Fibs: fibrillation potentials, MFCV: muscle fiber conduction velocity, MUP: motor unit potentials
* if motor unit action potentials (MUAP) showed polyphasia and marked reduction in duration (<8 ms) and height (<1000 microV) in >2 muscles, and spontaneous activity in ≥2 muscles
Magnetic resonance imaging (MRI)
As described above, Lehmann Urban (2021) included 191 patients in whom – in addition to EMG – an MRI was performed. MRI results (atrophy, fatty replacement, edema and contrast uptake) were presented for the diagnostic group “IIM”.
Van de Vlekkert (2015) included 48 patients of the 82 who were screened, because 19 did not meet the inclusion criteria and 15 met the exclusion criteria. In 35 out of 48 patients, IIM was diagnosed. The diagnostic accuracies of MRI by itself, MRI as a triage test for muscle biopsy, and MRI as add-on to muscle biopsy (if one or both tests are positive) were evaluated.
Of the 35 patients included by Weber (2006), 11 had confirmed PM (n = 3) or DM (n = 8). All but one underwent MRI; one patient with confirmed DM did not undergo MRI due to claustrophobia.
The sensitivity, specificity, and positive predictive value of MRI are depicted in table 2.
|
Study |
Test |
Sensitivity (95%CI) |
Specificity (95%CI) |
Positive predictive value (95%CI) |
|
Lehmann Urban (2021) |
MRI – atrophy |
0.24 (0.13 to 0.37) |
0.70 (0.62 to 0.77) |
0.22 (0.11 to 0.33) |
|
MRI – fatty replacement |
0.61 (0.46 to 0.74) |
0.30 (0.23 to 0.38) |
0.24 (0.17 to 0.31) |
|
|
MRI – oedema |
0.47 (0.33 to 0.62) |
0.50 (0.41 to 0.59) |
0.26 (0.17 to 0.34) |
|
|
MRI – contrast uptake |
0.29 (0.17 to 0.44) |
0.61 (0.52 to 0.69) |
0.21 (0.12 to 0.31) |
|
|
Van de Vlekkert (2015) |
MRI |
0.91 (0.77 to 0.98) |
0.69 (0.39 to 0.91) |
0.89 (0.79 to 0.99) |
|
MRI as triage for biopsy |
0.81 (0.64 to 0.93) |
1.00 (0.40 to 1.00) |
1.00 (0.40 to 1.00) |
|
|
MRI as add-on to biopsy |
0.94 (0.81 to 0.99) |
0.67 (0.35 to 0.90) |
0.89 (0.79 to 0.99) |
|
|
Weber (2006) |
MRI- oedema |
1.00 (0.69 to 1.00) |
0.88 (0.68 to 0.97) |
0.77 (0.54 to 0.99) |
Table 2. diagnostic measures for (features measured on) MRI for patients suspected of (inflammatory) myopathies. MRI: magnetic resonance imaging.
Ultrasound
In addition to MRI, the 35 patients from Weber’s (2006) study, underwent CEUS. Based on the obtained data, threshold values for US volume of 10.8 ml, for US blood flow of 5.8 ml/min/100g tissue, and for US blood flow velocity of 0.9 mm/s were determined. The diagnostic accuracy of these parameters for muscle perfusion are shown in table 3.
|
Study |
Test |
Sensitivity (95%CI) |
Specificity (95%CI) |
Positive predictive value (95%CI) |
|
Weber (2006) |
US – volume |
0.64 (0.31 to 0.89) |
0.88 (0.68 to 0.97) |
0.70 (0.42 to 0.98) |
|
US – flow |
0.73 (0.39 to 0.94) |
0.92 (0.73 to 0.99) |
0.80 (0.55 to 1.00) |
|
|
US – velocity |
0.36 ( 0.11 to 0.69) |
0.96 (0.79 to 1.00) |
0.80 (0.45 to 1.00) |
Table 3. diagnostic measures for muscle perfusion parameters, measured on contrast-enhances ultrasound, for patients suspected of polymyositis or dermatomyositis. US: ultrasound.
Level of evidence of the literature
EMG
The level of evidence regarding sensitivity, specificity and positive predictive value for EMG of MFCV measurement was downgraded by 2 levels to low because of unclear or unobjectifiable thresholds for the index test, and unclear exclusion criteria in some studies (-1, risk of bias), and because of estimates with large confidence intervals (-1, imprecision).
MRI
The level of evidence regarding sensitivity, specificity and positive predictive value for (specific characteristics of) MRI was downgraded by 2 levels to low:
- For MRI in general, MRI as add-on test, and MRI as triage test because of the inclusion of a single study with a very low number of patients (-2, imprecision)
- For oedema on MRI because of unclear in- and exclusion criteria and interpretation of index test with knowledge of the reference test results (-1, risk of bias); and large differences in diagnostic accuracy estimates without overlap of confidence intervals (-1, inconsistency)
- For atrophy, fatty replacement and contrast uptake on MRI because of unclear in- and exclusion criteria and possible interpretation of index test with knowledge of the reference test results (-1, risk of bias); and the inclusion of a single study with a low number of patients (-1, imprecision)
Ultrasound
The level of evidence regarding sensitivity, specificity and positive predictive value for muscle perfusion parameters measured through CEUS was downgraded by 3 levels to very low due to the inclusion of a single study, with a small number of included patients, with large confidence intervals (-1, imprecision), and a patient population in which already a strong suspicion for IIM existed after extensive neurological and dermatological testing causing possible overestimation of sensitivity. Additionally, risk of bias occurred due to unclear patient selection methods, the absence of the use of pre-specified thresholds for the index test, unclear reference test assessor blinding of results of the index test, and possible overoptimistic results because of the lack of funding information (-2, risk of bias).
Zoeken en selecteren
Search and select
A systematic review of the literature was performed to answer the following question:
What is the diagnostic test accuracy of different diagnostic modalities in patients with suspected IIM?
Scope: as these are addressed in other modules of this guideline, the following subjects will not be addressed in this module: diagnosing interstitial lung disease (ILD), screening for malignancies, and the diagnostic value of antibody detection. Here, only imaging modalities will be described.
P: patients in whom any form of IIM is suspected (including juvenile DM (JDM) and inclusion body myositis (IBM))
I: medical imaging (EMG, MRI, CT scan, US)
C: other diagnostic modalities from I
R: muscle biopsy
O: sensitivity, specificity, positive and negative predictive value
Timing and setting: during suspicion of IIM, usually in hospitals or neuromuscular outpatient clinics
Relevant outcome measures
The guideline development group considered specificity and positive predictive value as critical outcome measures for decision making; and sensitivity and negative predictive value as important outcome measures for decision making.
The working group defined minimal clinically important values for diagnostic accuracy measures (based on Šimundić, 2009):
- 90% or more is considered a good performance of the diagnostic test;
- 70% to 90% is a reasonable performance and might have potential clinical relevance
Search and select (Methods)
The databases Medline (via OVID) and Embase (via Embase.com) were searched using relevant search terms until November 30th, 2021. The detailed search strategy is depicted under the tab Methods.
The systematic literature search resulted in 795 hits. Studies were selected based on the following criteria:
- a) study design (systematic reviews, randomized controlled trials, and cohort studies);
- (b) study population (patients suspected of having IIM);
- (c) index test (any imaging modality);
- (d) reference test (muscle biopsy);
- (e) language (English or Dutch);
- (f) publication date (between 2002 and November 2021); and
- (g) availability of full text.
As the diagnostic value of antibody testing is assessed in a separate literature analysis (with appropriate search), studies evaluating the value of myositis specific antibodies will not be included for assessment.
Articles were screened based on title and abstract. This resulted in 24 articles. After reading the full text, five articles were eligible for inclusion. A table with reasons for exclusion is presented under the tab Methods.
Results
Five studies were included in the analysis of the literature for diagnostic modalities for IIM. No studies were included for JDM or IBM. 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
- Abdelnaby R, Mohamed KA, Elgenidy A, Sonbol YT, Bedewy MM, Aboutaleb AM, Ebrahim MA, Maallem I, Dardeer KT, Heikal HA, Gawish HM, Zschüntzsch J. Muscle Sonography in Inclusion Body Myositis: A Systematic Review and Meta-Analysis of 944 Measurements. Cells. 2022 Feb 9;11(4):600. doi: 10.3390/cells11040600. PMID: 35203250; PMCID: PMC8869828.
- Ahmed S, Concha JSS, Chakka S, Krain RL, Zamalin D, Foulke G, Werth VP. Diagnosing muscle disease in a cohort of classic dermatomyositis patients seen at a rheumatologic dermatology outpatient clinic. J Am Acad Dermatol. 2022 Mar;86(3):544-550. doi: 10.1016/j.jaad.2021.05.026. Epub 2021 May 26. PMID: 34051316.
- Allenbach Y, Benveniste O, Goebel HH, Stenzel W. Integrated classification of inflammatory myopathies. Neuropathol Appl Neurobiol. 2017 Feb;43(1):62-81. doi: 10.1111/nan.12380. PMID: 28075491.
- Blijham PJ, Hengstman GJ, Ter Laak HJ, Van Engelen BG, Zwarts MJ. Muscle-fiber conduction velocity and electromyography as diagnostic tools in patients with suspected inflammatory myopathy: a prospective study. Muscle Nerve. 2004 Jan;29(1):46-50. doi: 10.1002/mus.10519. PMID: 14694497.
- Cox FM, Reijnierse M, van Rijswijk CS, Wintzen AR, Verschuuren JJ, Badrising UA. Magnetic resonance imaging of skeletal muscles in sporadic inclusion body myositis. Rheumatology (Oxford). 2011 Jun;50(6):1153-61. doi: 10.1093/rheumatology/ker001. Epub 2011 Feb 2. PMID: 21288962.
- Furuta M, Furuta N, Nagashima K, Fujita Y, Tsushima Y, Ikeda Y. Differential and quantitative neuroimaging characteristics of inclusion body myositis. J Clin Neurosci. 2020 Feb;72:244-251. doi: 10.1016/j.jocn.2019.11.029. Epub 2019 Dec 12. PMID: 31839382.
- Greenberg SA. Inclusion Body Myositis. Continuum (Minneap Minn). 2016 Dec;22(6, Muscle and Neuromuscular Junction Disorders):1871-1888. doi: 10.1212/01.CON.0000511071.58338.1e. PMID: 27922498.
- Leeuwenberg KE, van Alfen N, Christopher-Stine L, Paik JJ, Tiniakou E, Mecoli C, Doorduin J, Saris CGJ, Albayda J. Ultrasound can differentiate inclusion body myositis from disease mimics. Muscle Nerve. 2020 Jun;61(6):783-788. doi: 10.1002/mus.26875. Epub 2020 Apr 11. PMID: 32239702; PMCID: PMC7317807.
- Lehmann Urban D, Mohamed M, Ludolph AC, Kassubek J, Rosenbohm A. The value of qualitative muscle MRI in the diagnostic procedures of myopathies: a biopsy-controlled study in 191 patients. Ther Adv Neurol Disord. 2021 Feb 26;14:1756286420985256. doi: 10.1177/1756286420985256. PMID: 33737953; PMCID: PMC7934066.
- Malartre S, Bachasson D, Mercy G, Sarkis E, Anquetil C, Benveniste O, Allenbach Y. MRI and muscle imaging for idiopathic inflammatory myopathies. Brain Pathol. 2021 May;31(3):e12954. doi: 10.1111/bpa.12954. PMID: 34043260; PMCID: PMC8412099.
- Nederlandse Vereniging voor Neurologie (NVN) en Interuniversitair Steunpunt Neuromusculair Onderzoek (ISNO). Richtlijn Dermatomyositis, polymyositis en sporadische 'inclusion body'-myositis. Hoofdstuk 3: Diagnostiek van volwassenen. 2005. Beschikbaar via: https://www.huidziekten.nl/richtlijnen/richtlijn-dermatomyositis-2005.pdf
- Noto Y, Shiga K, Tsuji Y, Kondo M, Tokuda T, Mizuno T, Nakagawa M. Contrasting echogenicity in flexor digitorum profundus-flexor carpi ulnaris: a diagnostic ultrasound pattern in sporadic inclusion body myositis. Muscle Nerve. 2014 May;49(5):745-8. doi: 10.1002/mus.24056. PMID: 24037920.
- Ross L, McKelvie P, Reardon K, Wong H, Wicks I, Day J. Muscle biopsy practices in the evaluation of neuromuscular disease: A systematic literature review. Neuropathol Appl Neurobiol. 2023 Feb;49(1):e12888. doi: 10.1111/nan.12888. PMID: 36734037.
- Sener U, Martinez-Thompson J, Laughlin RS, Dimberg EL, Rubin DI. Needle electromyography and histopathologic correlation in myopathies. Muscle Nerve. 2019 Mar;59(3):315-320. doi: 10.1002/mus.26381. Epub 2018 Dec 29. PMID: 30414326.
- Shenavandeh S, Rashidi F. Nailfold capillaroscopy changes with disease activity in patients with inflammatory myositis including overlap myositis, pure dermatomyositis, and pure polymyositis. Reumatologia. 2022;60(1):42-52. doi: 10.5114/reum.2022.114109. Epub 2022 Feb 28. PMID: 35645422; PMCID: PMC9132111.
- Šimundić AM. Measures of Diagnostic Accuracy: Basic Definitions. EJIFCC. 2009 Jan 20;19(4):203-11. PMID: 27683318; PMCID: PMC4975285.
- Tasca G, Monforte M, De Fino C, Kley RA, Ricci E, Mirabella M. Magnetic resonance imaging pattern recognition in sporadic inclusion-body myositis. Muscle Nerve. 2015 Dec;52(6):956-62. doi: 10.1002/mus.24661. Epub 2015 Aug 31. PMID: 25808807.
- Udd B, Stenzel W, Oldfors A, Olivé M, Romero N, Lammens M, Kusters B, Sewry C, Goebel HH, Evangelista T. 1st ENMC European meeting: The EURO-NMD pathology working group Recommended Standards for Muscle Pathology Amsterdam, The Netherlands, 7 December 2018. Neuromuscul Disord. 2019 Jun;29(6):483-485. doi: 10.1016/j.nmd.2019.03.002. Epub 2019 Mar 15. PMID: 31101462.
- Van De Vlekkert J, Maas M, Hoogendijk JE, De Visser M, Van Schaik IN. Combining MRI and muscle biopsy improves diagnostic accuracy in subacute-onset idiopathic inflammatory myopathy. Muscle Nerve. 2015 Feb;51(2):253-8. doi: 10.1002/mus.24307. PMID: 24895239.
- Weber MA, Jappe U, Essig M, Krix M, Ittrich C, Huttner HB, Meyding-Lamadé U, Hartmann M, Kauczor HU, Delorme S. Contrast-enhanced ultrasound in dermatomyositis- and polymyositis. J Neurol. 2006 Dec;253(12):1625-32. doi: 10.1007/s00415-006-0318-5. Erratum in: J Neurol. 2007 Sep;254(9):1308. Huttner, B Hagen (corrected to Huttner, Hagen B). PMID: 17219033.
- Wienke J, Bellutti Enders F, Lim J, Mertens JS, van den Hoogen LL, Wijngaarde CA, Yeo JG, Meyer A, Otten HG, Fritsch-Stork RDE, Kamphuis SSM, Hoppenreijs EPAH, Armbrust W, van den Berg JM, Hissink Muller PCE, Tekstra J, Hoogendijk JE, Deakin CT, de Jager W, van Roon JAG, van der Pol WL, Nistala K, Pilkington C, de Visser M, Arkachaisri T, Radstake TRDJ, van der Kooi AJ, Nierkens S, Wedderburn LR, van Royen-Kerkhof A, van Wijk F. Galectin-9 and CXCL10 as Biomarkers for Disease Activity in Juvenile Dermatomyositis: A Longitudinal Cohort Study and Multicohort Validation. Arthritis Rheumatol. 2019 Aug;71(8):1377-1390. doi: 10.1002/art.40881. Epub 2019 Mar 12. PMID: 30861625; PMCID: PMC6973145.
Evidence tabellen
Abbreviations (alphabetical): (CE)US: (contrast-enhanced) ultrasound, CI: confidence interval, CK: creatine kinase, DM: dermatomyositis, EMG: electromyography, ENMC: European Neuromuscular Centre, Fibs: fibrillation potentials, F/S ratio: fastest to slowest fiber, IBM: inclusion body myositis, IIM: idiopathic inflammatory myopathy, IQR: interquartile range, MFCV: Muscle fiber conduction velocity, MRI: magnetic resonance imaging, MU(A)P: motor unit (action) potentials, NAM: necrotizing autoimmune myopathy , NGS: next generation sequencing, NSM: non-specific myositis, PM: polymyositis, ROC: receiver operating characteristic.
Risk of bias table
|
Study reference |
Patient selection |
Index test |
Reference standard |
Flow and timing |
Comments with respect to applicability |
|
Blijham, 2004 |
Was a consecutive or random sample of patients enrolled? Yes
Was a case-control design avoided? Semi: all patients with suspected IIM were included as a retrospective cohort, but compared to healthy controls of whom selection was unclear.
Did the study avoid inappropriate exclusions? Unclear: exclusion criteria not described |
Were the index test results interpreted without knowledge of the results of the reference standard? Unclear: index test performed before reference standard, but unclear whether cut-offs were calculated with knowledge of the reference standard
If a threshold was used, was it pre-specified? No
|
Is the reference standard likely to correctly classify the target condition? Yes
Were the reference standard results interpreted without knowledge of the results of the index test? Unclear
|
Was there an appropriate interval between index test(s) and reference standard? Yes
Did all patients receive a reference standard? Yes
Did patients receive the same reference standard? Yes
Were all patients included in the analysis? No: two were excluded due to insufficient muscle fiber potentials to calculate MFCV |
Are there concerns that the included patients do not match the review question? No
Are there concerns that the index test, its conduct, or interpretation differ from the review question? No
Are there concerns that the target condition as defined by the reference standard does not match the review question? No |
|
CONCLUSION: Could the selection of patients have introduced bias? RISK: HIGH |
CONCLUSION: Could the conduct or interpretation of the index test have introduced bias? RISK: HIGH |
CONCLUSION: Could the reference standard, its conduct, or its interpretation have introduced bias? RISK: UNCLEAR |
CONCLUSION Could the patient flow have introduced bias? RISK: LOW |
||
|
Lehmann Urban, 2021 |
Was a consecutive or random sample of patients enrolled? No
Was a case-control design avoided? Yes
Did the study avoid inappropriate exclusions? Unclear: inclusion- and exclusion criteria not reported
|
Were the index test results interpreted without knowledge of the results of the reference standard? Yes: at least for EMG. Unclear for MRI.
If a threshold was used, was it pre-specified? Yes, but limitedly objectifiable
|
Is the reference standard likely to correctly classify the target condition? Yes
Were the reference standard results interpreted without knowledge of the results of the index test? Probably no: index testing occurred before “reference testing”, possibly aiding in making diagnosis
|
Was there an appropriate interval between index test(s) and reference standard? Yes
Did all patients receive a reference standard? Yes
Did patients receive the same reference standard? Yes
Were all patients included in the analysis? Yes |
Are there concerns that the included patients do not match the review question? Possibly, patients were referred for suspected myopathy, not specifically for inflammatory myopathy
Are there concerns that the index test, its conduct, or interpretation differ from the review question? No
Are there concerns that the target condition as defined by the reference standard does not match the review question? No |
|
CONCLUSION: Could the selection of patients have introduced bias? RISK: HIGH |
CONCLUSION: Could the conduct or interpretation of the index test have introduced bias? RISK: LOW |
CONCLUSION: Could the reference standard, its conduct, or its interpretation have introduced bias? RISK: UNCLEAR |
CONCLUSION Could the patient flow have introduced bias? RISK: LOW |
||
|
Sener, 2019 |
Was a consecutive or random sample of patients enrolled? Yes
Was a case-control design avoided? Yes
Did the study avoid inappropriate exclusions? Yes
|
Were the index test results interpreted without knowledge of the results of the reference standard? Yes
If a threshold was used, was it pre-specified? Unclear
|
Is the reference standard likely to correctly classify the target condition? Yes: muscle biopsy and follow-up based on review of medical record notes.
Were the reference standard results interpreted without knowledge of the results of the index test? No
|
Was there an appropriate interval between index test(s) and reference standard? Yes
Did all patients receive a reference standard? Yes
Did patients receive the same reference standard? Yes
Were all patients included in the analysis? Yes (except for those excluded based on specified exclusion criteria). |
Are there concerns that the included patients do not match the review question? Possibly, patients were referred for muscle biopsy for suspected myopathy, not specifically for inflammatory myopathy
Are there concerns that the index test, its conduct, or interpretation differ from the review question? No
Are there concerns that the target condition as defined by the reference standard does not match the review question? No. |
|
CONCLUSION: Could the selection of patients have introduced bias? RISK: LOW |
CONCLUSION: Could the conduct or interpretation of the index test have introduced bias? RISK: HIGH |
CONCLUSION: Could the reference standard, its conduct, or its interpretation have introduced bias? RISK: LOW |
CONCLUSION Could the patient flow have introduced bias? RISK: LOW |
||
|
Van de Vlekkert, 2015 |
Was a consecutive or random sample of patients enrolled? Yes
Was a case-control design avoided? Yes
Did the study avoid inappropriate exclusions? Yes; yet patients with high suspicion for sporadic IBM were excluded
|
Were the index test results interpreted without knowledge of the results of the reference standard? Yes
If a threshold was used, was it pre-specified? Yes, yet not fully objectifiable
|
Is the reference standard likely to correctly classify the target condition? Yes: response to corticosteroids during follow-up in addition to European criteria
Were the reference standard results interpreted without knowledge of the results of the index test? No: MRI aided in making diagnosis
|
Was there an appropriate interval between index test(s) and reference standard? Yes
Did all patients receive a reference standard? Yes
Did patients receive the same reference standard? Yes
Were all patients included in the analysis? Yes (except for those excluded based on specified exclusion criteria).For 1 patient missing data on muscle biopsy. |
Are there concerns that the included patients do not match the review question? No
Are there concerns that the index test, its conduct, or interpretation differ from the review question? No
Are there concerns that the target condition as defined by the reference standard does not match the review question? No |
|
CONCLUSION: Could the selection of patients have introduced bias? RISK: LOW |
CONCLUSION: Could the conduct or interpretation of the index test have introduced bias? RISK: LOW |
CONCLUSION: Could the reference standard, its conduct, or its interpretation have introduced bias? RISK: LOW |
CONCLUSION Could the patient flow have introduced bias? RISK: LOW |
||
|
Weber, 2006 |
Was a consecutive or random sample of patients enrolled? No
Was a case-control design avoided? Yes
Did the study avoid inappropriate exclusions? Unclear: inclusion- and exclusion criteria not sufficiently mentioned.
|
Were the index test results interpreted without knowledge of the results of the reference standard? No, threshold values were calculated based on muscle histology results.
If a threshold was used, was it pre-specified? No, calculated based on obtained data.
|
Is the reference standard likely to correctly classify the target condition? Yes
Were the reference standard results interpreted without knowledge of the results of the index test? Unclear whether assessor was blinded for CEUS results
|
Was there an appropriate interval between index test(s) and reference standard? Yes
Did all patients receive a reference standard? Yes
Did patients receive the same reference standard? Yes
Were all patients included in the analysis? Yes |
Are there concerns that the included patients do not match the review question? No, yet patients in this study have a much higher suspicion of IIM than in the other studies, due to extensive testing before inclusion.
Are there concerns that the index test, its conduct, or interpretation differ from the review question? No, yet it is unclear how often muscle perfusion parameters are measured in daily practice.
Are there concerns that the target condition as defined by the reference standard does not match the review question? No |
|
|
CONCLUSION: Could the selection of patients have introduced bias? RISK: HIGH |
CONCLUSION: Could the conduct or interpretation of the index test have introduced bias? RISK: HIGH |
CONCLUSION: Could the reference standard, its conduct, or its interpretation have introduced bias? RISK: UNCLEAR |
CONCLUSION Could the patient flow have introduced bias? RISK: LOW |
Abbreviations: CEUS: contrast-enhanced ultrasound, EMG: electromyography, IIM: idiopathic inflammatory myopathy, MFCV: Muscle fiber conduction velocity, MRI: magnetic resonance imaging
Table of excluded studies
|
Reference |
Reason for exclusion |
|
Aburahma SK, Wicklund MP, Quan D. Take two: Utility of the repeat skeletal muscle biopsy. Muscle Nerve. 2019 Jul;60(1):41-46. doi: 10.1002/mus.26484. Epub 2019 Apr 22. PMID: 30972775. |
Wrong intervention of interest (repeat muscle biopsy) |
|
Ahmed S, Concha JSS, Chakka S, Krain RL, Zamalin D, Foulke G, Werth VP. Diagnosing muscle disease in a cohort of classic dermatomyositis patients seen at a rheumatologic dermatology outpatient clinic. J Am Acad Dermatol. 2022 Mar;86(3):544-550. doi: 10.1016/j.jaad.2021.05.026. Epub 2021 May 26. PMID: 34051316. |
Wrong study design |
|
An YS, Suh CH, Jung JY, Kim HA. Role of bone scan in the assessment of polymyositis/dermatomyositis. Clin Rheumatol. 2015 Apr;34(4):699-706. doi: 10.1007/s10067-014-2837-2. Epub 2014 Dec 12. PMID: 25501462. |
Wrong study design |
|
Challa S, Jakati S, Narla S, Uppin MS, Kannan MA, Jagarlapudi MKM. Sporadic Inclusion Body Myositis: A Clinicopathological Study. Neurol India. 2021 May-Jun;69(3):638-641. doi: 10.4103/0028-3886.319212. PMID: 34169859. |
Wrong study design |
|
Connor A, Stebbings S, Anne Hung N, Hammond-Tooke G, Meikle G, Highton J. STIR MRI to direct muscle biopsy in suspected idiopathic inflammatory myopathy. J Clin Rheumatol. 2007 Dec;13(6):341-5. doi: 10.1097/RHU.0b013e31815dca0a. PMID: 18176145. |
Wrong study design |
|
Cox FM, Reijnierse M, van Rijswijk CS, Wintzen AR, Verschuuren JJ, Badrising UA. Magnetic resonance imaging of skeletal muscles in sporadic inclusion body myositis. Rheumatology (Oxford). 2011 Jun;50(6):1153-61. doi: 10.1093/rheumatology/ker001. Epub 2011 Feb 2. PMID: 21288962. |
Wrong study design |
|
Didona D, Juratli HA, Scarsella L, Keber U, Eming R, Hertl M. Amyopathic and anti-TIF1 gamma-positive dermatomyositis: analysis of a monocentric cohort and proposal to update diagnostic criteria. Eur J Dermatol. 2020 Jun 1;30(3):279-288. doi: 10.1684/ejd.2020.3766. PMID: 32666928. |
Wrong study design |
|
Fornaro M, Girolamo F, Cavagna L, Franceschini F, Giannini M, Amati A, Lia A, Tampoia M, D'Abbicco D, Maggi L, Fredi M, Zanframundo G, Moschetti L, Coladonato L, Iannone F. Severe muscle damage with myofiber necrosis and macrophage infiltrates characterize anti-Mi2 positive dermatomyositis. Rheumatology (Oxford). 2021 Jun 18;60(6):2916-2926. doi: 10.1093/rheumatology/keaa739. PMID: 33249503. |
Wrong study design |
|
Haczkiewicz K, Sebastian A, Piotrowska A, Misterska-Skóra M, Hałoń A, Skoczyńska M, Sebastian M, Wiland P, Dzięgiel P, Podhorska-Okołów M. Immunohistochemical and ultrastructural analysis of sporadic inclusion body myositis: a case series. Rheumatol Int. 2019 Jul;39(7):1291-1301. doi: 10.1007/s00296-018-4221-z. Epub 2018 Dec 8. PMID: 30535925. |
Wrong study design |
|
Ikenaga C, Findlay AR, Goyal NA, Robinson S, Cauchi J, Hussain Y, Wang LH, Kershen JC, Beson BA, Wallendorf M, Bucelli RC, Mozaffar T, Pestronk A, Weihl CC. Clinical utility of anti-cytosolic 5'-nucleotidase 1A antibody in idiopathic inflammatory myopathies. Ann Clin Transl Neurol. 2021 Mar;8(3):571-578. doi: 10.1002/acn3.51294. Epub 2021 Feb 8. PMID: 33556224; PMCID: PMC7951108. |
Wrong study design |
|
Larman HB, Salajegheh M, Nazareno R, Lam T, Sauld J, Steen H, Kong SW, Pinkus JL, Amato AA, Elledge SJ, Greenberg SA. Cytosolic 5'-nucleotidase 1A autoimmunity in sporadic inclusion body myositis. Ann Neurol. 2013 Mar;73(3):408-18. doi: 10.1002/ana.23840. PMID: 23596012. |
Wrong study design |
|
Lim J, Eftimov F, Raaphorst J, Aronica E, van der Kooi AJ. Diagnostic value of additional histopathological fascia examination in idiopathic inflammatory myopathies. Eur J Neurol. 2019 Dec;26(12):1494-1496. doi: 10.1111/ene.14027. Epub 2019 Jul 18. PMID: 31220379; PMCID: PMC6916207. |
Wrong study design |
|
Montagnese F, Babačić H, Eichhorn P, Schoser B. Evaluating the diagnostic utility of new line immunoassays for myositis antibodies in clinical practice: a retrospective study. J Neurol. 2019 Jun;266(6):1358-1366. doi: 10.1007/s00415-019-09266-4. Epub 2019 Mar 6. PMID: 30840145. |
Described myositis specific antibody testing (included in other analysis) |
|
Nagawa K, Suzuki M, Yamamoto Y, Inoue K, Kozawa E, Mimura T, Nakamura K, Nagata M, Niitsu M. Texture analysis of muscle MRI: machine learning-based classifications in idiopathic inflammatory myopathies. Sci Rep. 2021 May 10;11(1):9821. doi: 10.1038/s41598-021-89311-3. PMID: 33972636; PMCID: PMC8110584. |
Described intervention not routine practice in the Netherlands |
|
Nojszewska M, Gawel M, Kierdaszuk B, Sierdziński J, Szmidt-Sałkowska E, Seroka A, Kamińska AM, Kostera-Pruszczyk A. Electromyographic findings in sporadic inclusion body myositis. J Electromyogr Kinesiol. 2018 Apr;39:114-119. doi: 10.1016/j.jelekin.2018.02.003. Epub 2018 Feb 11. PMID: 29482084. |
Wrong study design |
|
Paramalingam S, Counsel P, Mastaglia FL, Keen H, Needham M. Imaging in the diagnosis of idiopathic inflammatory myopathies; indications and utility. Expert Rev Neurother. 2019 Feb;19(2):173-184. doi: 10.1080/14737175.2019.1572507. Epub 2019 Feb 7. PMID: 30661408. |
Wrong study design |
|
Sag E, Demir S, Bilginer Y, Talim B, Haliloglu G, Topaloglu H, Ozen S. Clinical features, muscle biopsy scores, myositis specific antibody profiles and outcome in juvenile dermatomyositis. Semin Arthritis Rheum. 2021 Feb;51(1):95-100. doi: 10.1016/j.semarthrit.2020.10.007. Epub 2020 Dec 22. PMID: 33360233. |
Wrong study design |
|
Ukichi T, Yoshida K, Matsushima S, Kawakami G, Noda K, Furuya K, Kurosaka D. MRI of skeletal muscles in patients with idiopathic inflammatory myopathies: characteristic findings and diagnostic performance in dermatomyositis. RMD Open. 2019 Mar 28;5(1):e000850. doi: 10.1136/rmdopen-2018-000850. PMID: 30997152; PMCID: PMC6443133. |
Wrong study design |
|
Yang F, Jing F, Chen Z, Ling L, Wang R, Wang X, Tin C, Pu C, Shi X, Huo Y, Zhang X, Huang X, Yu S. Electrophysiological and clinical examination of polymyositis: a retrospective analysis. Am J Med Sci. 2014 Aug;348(2):162-6. doi: 10.1097/MAJ.0000000000000216. PMID: 24556930. |
Wrong study design |
Verantwoording
Autorisatiedatum en geldigheid
Laatst beoordeeld : 07-02-2024
Laatst geautoriseerd : 07-02-2024
Geplande herbeoordeling :
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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