Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2018 May;14(5):303-318.
doi: 10.1038/nrrheum.2018.33. Epub 2018 Apr 12.

Update on outcome assessment in myositis

Lisa G Rider  1 , Rohit Aggarwal  2 , Pedro M Machado  3 , Jean-Yves Hogrel  4 , Ann M Reed  5 , Lisa Christopher-Stine  6 , Nicolino Ruperto  7
Affiliations
Review

Update on outcome assessment in myositis

Lisa G Rider et al. Nat Rev Rheumatol. 2018 May.

Abstract

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.

PubMed Disclaimer

Conflict of interest statement

Competing interests.

The authors declare no competing interests.

Figures

Figure 1
Figure 1
Fat-fraction T1 magnetic resonance imaging (MRI) map in inclusion body myositis. Fat-fraction T1 MRI map of the right thigh in a patient with inclusion body myositis, at baseline and one-year follow-up. An absolute percentage increase of 6.2% in fat fraction was observed.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Miller FW et al. for the International Myositis Outcome Assessment Collaborative Study Group. Proposed preliminary core set measures for disease outcome assessment in adult and juvenile idiopathic inflammatory myopathies. Rheumatology (Oxford) 40, 1262–1273 (2001). - PubMed
    1. Ruperto N & Martini A Networking in paediatrics: the example of the Paediatric Rheumatology International Trials Organisation (PRINTO). Arch. Dis. Child 96, 596–601 (2011). - PubMed
    1. Rider LG et al. 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 Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI). Arthritis Care Res. (Hoboken.) 63 Suppl 11, S118–S157 (2011). - PMC - PubMed
    1. Ruperto N et al. The provisional Paediatric Rheumatology International Trials Organisation/American College of Rheumatology/European League Against Rheumatism Disease activity core set for the evaluation of response to therapy in juvenile dermatomyositis: a prospective validation study. Arthritis Rheum 59, 4–13 (2008). - PubMed
    1. Rose MR 188th ENMC International Workshop: Inclusion Body Myositis, 2–4 December 2011, Naarden, The Netherlands. Neuromuscul. Disord 23, 1044–1055 (2013). - PubMed

Publication types