2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria—methodological aspects

Rider, Lisa G.; Ruperto, Nicolino; Pistorio, Angela; Erman, Brian; Bayat, Nastaran; Lachenbruch, Peter A.; Rockette, Howard E.; Feldman, Brian M.; Huber, Adam M.; Hansen, Paul; Oddis, Chester V.; Lundberg, Ingrid E.; Amato, Anthony A.; Chinoy, Hector; Cooper, Robert G.; Chung, Lorinda; Dankó, Katalin; Fiorentino, David; Torre, Ignacio García‐De La; Reed, Ann M.; Song, Yeong Wook; Cimaz, Rolando; Cuttica, Rubén; Pilkington, Clarissa; Martini, Alberto; Net, Janjaap van der; Maillard, Susan; Miller, Frederick W.; Vencovský, Jiří; Aggarwal, Rohit

doi:10.1093/rheumatology/kex226

Cited by 35 publications

(23 citation statements)

References 36 publications

(29 reference statements)

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“…The CSMs of disease activity have also been responsive to changes in disease activity, as demonstrated in several recent therapeutic trials for JDM and adult DM/PM 6–10 . The degree of change in each disease activity measure that is clinically significant has also been established, with at least 20% improvement in all IMACS or PRINTO CSMs, except 30% improvement in muscle enzymes, considered as a minimally clinically meaningful degree of change; moderate and major changes in CSMs of activity have also been defined 11 . The relative degree of importance of each measure has been determined using conjoint analysis, with muscle strength being considered most important and physician global activity (PGA) followed by extramuscular activity as next in importance among the CSMs of disease activity for adult DM/PM and JDM 11 .…”

Section: Core Set Measuresmentioning

confidence: 99%

Update on outcome assessment in myositis

et al. 2018

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The adult and juvenile myositis syndromes, commonly referred to collectively as idiopathic inflammatory myopathies (IIMs), are systemic autoimmune diseases with the hallmarks of muscle weakness and inflammation. Validated, well-standardized measures to assess disease activity, known as core set measures, were developed by international networks of myositis researchers for use in clinical trials. Composite response criteria using weighted changes in the core set measures of disease activity were developed and validated for adult and juvenile patients with dermatomyositis and adult patients with polymyositis, with different thresholds for minimal, moderate and major improvement in adults and juveniles. Additional measures of muscle strength and function are being validated to improve content validity and sensitivity to change. A health-related quality of life measure, which incorporates patient input, is being developed for adult patients with IIM. Disease state criteria, including criteria for inactive disease and remission, are being used as secondary end points in clinical trials. MRI of muscle and immunological biomarkers are promising approaches to discriminate between disease activity and damage and might provide much-needed objective outcome measures. These advances in the assessment of outcomes for myositis treatment, along with collaborations between international networks, should facilitate further development of new therapies for patients with IIM.

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Section: Core Set Measuresmentioning

confidence: 99%

Update on outcome assessment in myositis

et al. 2018

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“…In our experience, a child may appear to have quiescent JDM, but gene data, including RNASeq [15] and the SomaScan® data presented here, suggest continued activation of cytokine and metabolic systems among others (see below). Recent international collaboration has produced criteria for adults and children with dermatomyositis for both disease status and improvement [16,17]; but, of note, both the parent and the physician global ratings are subjective.…”

Section: Introductionmentioning

confidence: 99%

Serum protein biomarkers for juvenile dermatomyositis: a pilot study

et al. 2020

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Background Blood accessible biomarkers to assess disease activity and their response to therapies in Juvenile Dermatomyositis (JDM) are urgently needed. This pilot study aims to identify serum protein biomarkers associated with clinical disease activity in untreated JDM and their response to medical therapy. Methods SomaScan® technology screened JDM patients for 1305 proteins at three points: 1) before start of treatment, 2) while on therapy, and 3) after treatment tapering when patients were clinically inactive. To define disease associated biomarkers, SomaScan® data from untreated JDM patients (n = 8) were compared to SomaScan® data from an independent age-matched healthy control group (n = 12). Longitudinal analysis defined treatment responsive proteins at three time points: untreated (7 samples), treated (7 samples), and clinically inactive (6 samples). To confirm the SomaScan® data, a subset of nine candidate proteins (CXCL11, IL-17B, IL-17D, IL-22, CXCL10, MCP-1, ANGPT2, MIF, IL-23) were tested by ELISA after adding 2 JDM (one untreated, one clinically inactive) serum samples to the same group of JDM girls (8 untreated, 7 treated; 7 clinically inactive) as well as with 17 age, gender, matched healthy controls. Results Comparison of untreated JDM versus healthy controls identified 202 elevated and 49 decreased serum proteins in JDM patients with an adjusted p-value < 0.001. Only 82 out of 251 identified biomarker candidates responded to treatment while 12 out of these 82 proteins returned to their original untreated disease levels upon therapy tapering. The ELISA testing of the untreated samples for nine candidate proteins confirmed previously known biomarkers (CXCL10 or IP-10, CXCL11 or I-TAC and MCP-1) and identified novel biomarkers including IL-22, Angiopoetin-2, and IL-17B in a cross-sectional analysis comparing 8 untreated JDM and 17 age/gender matched controls. The subsequent longitudinal data by ELISA were not concordant for some biomarkers (IL-22 and IL-17B), but the other biomarkers either normalized or rebounded concordantly. Conclusions Blood accessible protein biomarkers reflecting JDM pathophysiology were identified; some of them rebounded after therapy was tapered. Further studies bridging these biomarkers to specific clinical features of JDM are required to confirm the clinical utility of these serum protein biomarkers.

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“…The exclusion criteria were: severe kidney, liver or heart failure, as well as patients with systemic sclerosis since edema may be a feature at disease onset. IMNM, DM and anti-synthetase patients based on clinico-serological and/or clinico-pathological criteria (2,21,28) without clinical limb edema were included as controls.…”

Section: Patientsmentioning

confidence: 99%

“…Idiopathic inflammatory myopathies (IIM) are a heterogeneous condition involving the skeletal muscle but also displaying extra-muscular features. Recently, myositis-specific antibodies (MSA) have been identified and permitted to refine classification criteria (4,(21)(22)28). Using a clinico-serological and/or pathological approaches, IIM were classified into: dermatomyositis (DM), inclusion body myositis (IBM), and polymyositis (PM) (28) or immune-mediated necrotizing myopathy (IMNM), and anti-synthetase syndrome (ASyS) (22).…”

Section: Introductionmentioning

confidence: 99%

“…Recently, myositis-specific antibodies (MSA) have been identified and permitted to refine classification criteria (4,(21)(22)28). Using a clinico-serological and/or pathological approaches, IIM were classified into: dermatomyositis (DM), inclusion body myositis (IBM), and polymyositis (PM) (28) or immune-mediated necrotizing myopathy (IMNM), and anti-synthetase syndrome (ASyS) (22). Among the extra-muscular features, only the presence of a classical DM skin rash has been included as a classification criterion (21,28) yet other skin manifestations have been described in IIM.…”

Section: Introductionmentioning

confidence: 99%

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Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

et al. 2020

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Aims: Edema of the limbs is uncommon in idiopathic inflammatory myopathies (IIM). The few reported cases have been associated with severe and refractory dermatomyositis (DM), sometimes in association with cancers. We aimed to determine if edematous myositis is a homogeneous subtype based on clinical, serological and pathological features. Methods: This is a retrospective observational study performed between 2008 and 2015 in the French national referral center for myositis. All adult patients with an inflammatory muscle biopsy and upper limbs edema were included as well as IIM cases without limb edema as controls. Clinical, biological and pathological features were collected. Results: Seventeen edematous myositis were included and compared to 174 IIM without edema, including 50 DM controls. Edema was the first manifestation in 23% of patients. Muscle weakness was severe and symmetric, 71% of patients presented dysphagia and a restrictive ventilatory pattern was found in 40%. Fifty‐two percent of patients had a typical DM skin rash and 23% had cancer within 3 years of diagnosing myositis. Fifty‐three percent of patients presented a myositis specific antibody and only DM‐specific antibodies were detected. Classic pathological DM features (perifascicular atrophy, perifascicular/perimysial perivascular inflammation) were uncommon but capillary C5b‐9 deposition and MxA expression were seen in 79% and 73% of cases, respectively. A perimysial edema was found in 82% of cases. Seventeen percent of patients died (median follow up of 18 months). Edematous myositis demonstrated more marked capillary C5b‐9 deposition compared to IIM controls. There was no clinical, biological or pathological difference with DM controls except for limb edema. Conclusion: Our study underlines that limb edema could be a symptom of IIM and that edematous myositis are mostly DM. The vasculopathy seems to play a key role in its pathophysiology. Limb edema associated with muscle impairment should suggest the diagnosis of DM in clinical settings.

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2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria—methodological aspects

Abstract: Consensus methodology, with definitions tested on patient profiles and validated using clinical trials, led to 18 definitions for adult PM/DM and 14 for JDM as excellent candidates for consideration in the final consensus on new response criteria for myositis.

Cited by 35 publications

References 36 publications

Update on outcome assessment in myositis

Update on outcome assessment in myositis

Serum protein biomarkers for juvenile dermatomyositis: a pilot study

Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

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