Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: Physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C‐HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF‐36), Child Health Questionnaire (CHQ), Physician Global Damage, Myositis Damage Index (MDI), Quantitative Muscle

Rider, Lisa G.; Werth, Victoria P.; Huber, Adam M.; Alexanderson, Helene; Rao, Anand Prahalad; Ruperto, Nicolino; Herbelin, Laura; Barohn, Richard J.; Isenberg, David; Miller, Frederick W.

doi:10.1002/acr.20532

Cited by 325 publications

(207 citation statements)

References 99 publications

(397 reference statements)

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“…The medical research council (MRC) scale is the most widely accepted system to measure the strength level in individual muscles [38, 39]. However, the MRC scale has a limited capacity for documenting the full spectrum of strength in those with moderate weakness (i.e., those with an MRC strength score of 4, who have “active movement against gravity and resistance”).…”

Section: Diagnosis and Managementmentioning

confidence: 99%

Immune-Mediated Necrotizing Myopathy

2018

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Purpose of Review Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement. Here, we will review the characteristics of patients with IMNM. Recent Findings Anti-signal recognition particle (SRP) and anti-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) autoantibodies are closely associated with IMNM and define unique subtypes of patients. Importantly, the new European Neuromuscular Centre criteria recognize anti-SRP myopathy, anti-HMGCR myopathy, and autoantibody-negative IMNM as three distinct subtypes of IMNM. Anti-SRP myopathy patients have more severe muscle involvement, have more common extra-muscular features, and may respond best to immunosuppressive regimens that include rituximab. In contrast, anti-HMGCR myopathy is often associated with statin exposure and intravenous immunoglobulin treatment may be an effective treatment, even as monotherapy. Both anti-SRP and anti-HMGCR myopathy tend to be most severe in younger patients. Furthermore, children with these forms of IMNM may present with dystrophy-like features which are potentially reversible with immunosuppressant treatment. IMNM patients with either autoantibody may experience fatty replacement of muscle soon after disease onset, suggesting that intense and early immunosuppressant therapy may provide the best chance to avoid long-term disability. Summary IMNM is composed of anti-SRP myopathy, anti-HMGCR myopathy, and autoantibody-negative IMNM. Both anti-SRP and anti-HMGCR myopathy can cause severe weakness, especially in younger patients. Anti-SRP myopathy patients tend to have the most severe weakness and most prevalent extra-muscular features. Autoantibody-negative IMNM remains poorly described.

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Section: Diagnosis and Managementmentioning

confidence: 99%

Immune-Mediated Necrotizing Myopathy

2018

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“…Recent efforts have led to the production of the semivalidated International Myositis Assessment and Clinical Studies Group's (IMACS) 'core-set' outcome measures [Rider et al 2011] and 'definition of improvement' [Rider et al 2003]. These are increasingly utilized in clinical trials as primary outcome measures.…”

Section: Clinical Trials and The Therapeutic Evidence Basementioning

confidence: 99%

“…Not all measures are completely validated and some aspects exhibit a 'ceiling effect', especially in patients with relatively mild disease at the outset [Rider et al 2011]. Furthermore, differentiating between disease damage (i.e.…”

Section: Clinical Trials and The Therapeutic Evidence Basementioning

confidence: 99%

Selected aspects of the current management of myositis

Lilleker

Murphy

Cooper

2016

Therapeutic Advances in Musculoskeletal

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Abstract:The idiopathic inflammatory myopathies (IIM) are a rare and heterogeneous group of acquired autoimmune muscle disorders, often referred to as 'myositis'. Clinical assessment, together with muscle biopsy findings and autoantibody status are key factors to consider when making a diagnosis of IIM, and in stratification of the 'IIM spectrum' into disease subgroups. Treatment stratified according to serotype (and in the future, likely also genotype) is increasingly being used to take account of the heterogeneity within the IIM spectrum. Subgroup classification is also important in terms of monitoring for complications, such as malignancy and interstitial lung disease. Disease monitoring should include the use of standardized tools such as the IMACS disease activity outcome measures. Other tools such as muscle MRI can be useful in identifying areas of active muscle inflammation. Treatment outcomes in IIM remain unsatisfactory. The evidence base to guide treatment decisions is remarkably limited. In addition to muscle inflammation, a number of noninflammatory cellmediated mechanisms may contribute to weakness and disability, and for which no specific treatments are currently available.

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“…We have categorized this listing of myositis research registries into different groups (see Tables 1, 2, and 3) [13**,14,15*,16–28,29*,30*,31–37,38*,39–42,43* 44,45,46*,47–60,61**,62,63*,64*,65, 66*,67,68**,69*,70*,71,72*,73–82,83*,84–90,91*,92–107,108*,109,110**,111** 112–115,116*,117, 118*,119–121, 122**,123–129, 130*,131–133,134*,135,136*, 137–149,150*,151,152,153*,154*] based on the types of myositis included, as well as whether the registries are regional, national, or international in scope. Thirteen registries are international and 33 are national or regional registries.…”

Section: Myositis Registries and Biorepositoriesmentioning

confidence: 99%

“…Core set measures of myositis activity have been agreed upon to be assessed in all therapeutic trials for dermatomyositis, polymyositis, and JDM [101,119,123–126], and these have been combined to provide preliminary definitions of improvement that represent clinically meaningful change to be used as therapeutic trial endpoints [19,120,127]. These core set measures have also been utilized in preliminary definitions of moderate and major response clinical response, representing larger degrees of clinical improvement, and of clinically inactive disease for JDM [121,122**].…”

Section: Research Advances Through Myositis Registries and Biorepositmentioning

confidence: 99%

Myositis registries and biorepositories

Rider¹,

Dankó²,

Miller³

2014

Current Opinion in Rheumatology

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Purpose of review Clinical registries and biorepositories have proven extremely useful in many studies of diseases, especially rare diseases. Given their rarity and diversity, the idiopathic inflammatory myopathies, or myositis syndromes, have benefited from individual researchers’ collections of cohorts of patients. Major efforts are being made to establish large registries and biorepositories that will allow many additional studies to be performed that were not possible before. Here we describe the registries developed by investigators and patient support groups that are currently available for collaborative research purposes. Recent findings We have identified 46 myositis research registries, including many with biorepositories, which have been developed for a wide variety of purposes and have resulted in great advances in understanding the range of phenotypes, clinical presentations, risk factors, pathogenic mechanisms, outcome assessment, therapeutic responses, and prognoses. These are now available for collaborative use to undertake additional studies. Two myositis patient registries have been developed for research, and myositis patient support groups maintain demographic registries with large numbers of patients available to be contacted for potential research participation. Summary Investigator-initiated myositis research registries and biorepositories have proven extremely useful in understanding many aspects of these rare and diverse autoimmune diseases. These registries and biorepositories, in addition to those developed by myositis patient support groups, deserve continued support to maintain the momentum in this field as they offer major opportunities to improve understanding of the pathogenesis and treatment of these diseases in cost-effective ways.

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Cited by 325 publications

References 99 publications

Immune-Mediated Necrotizing Myopathy

Immune-Mediated Necrotizing Myopathy

Selected aspects of the current management of myositis

Myositis registries and biorepositories

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