The clinical phenotypes of the juvenile idiopathic inflammatory myopathies

Mona Shah; Gulnara Mamyrova; Ira N Targoff; Adam M Huber; James D Malley; Madeline Murguia Rice; Frederick W Miller; Lisa G Rider; Childhood Myositis Heterogeneity Collaborative Study Group

doi:10.1097/MD.0b013e31827f264d

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The clinical phenotypes of the juvenile idiopathic inflammatory myopathies

Journal article

Open access

The clinical phenotypes of the juvenile idiopathic inflammatory myopathies

Mona Shah, Gulnara Mamyrova, Ira N Targoff, Adam M Huber, James D Malley, Madeline Murguia Rice, Frederick W Miller, Lisa G Rider and Childhood Myositis Heterogeneity Collaborative Study Group

Medicine (Baltimore), Vol.92(1), pp.25-41

01/2013

DOI: 10.1097/MD.0b013e31827f264d

PMCID: PMC4580479

PMID: 23263716

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Abstract

The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with another autoimmune or connective tissue disease (JCTM), have not been well characterized, and their similarity to the adult clinical subgroups is unknown. We enrolled 436 patients with JIIM, including 354 classified as JDM, 33 as JPM, and 49 as JCTM, in a nationwide registry study. The aim of the study was to compare demographics; clinical features; laboratory measures, including myositis autoantibodies; and outcomes among these clinical subgroups, as well as with published data on adult patients with idiopathic inflammatory myopathies (IIM) enrolled in a separate natural history study. We used random forest classification and logistic regression modeling to compare clinical subgroups, following univariate analysis. JDM was characterized by typical rashes, including Gottron papules, heliotrope rash, malar rash, periungual capillary changes, and other photosensitive and vasculopathic skin rashes. JPM was characterized by more severe weakness, higher creatine kinase levels, falling episodes, and more frequent cardiac disease. JCTM had more frequent interstitial lung disease, Raynaud phenomenon, arthralgia, and malar rash. Differences in autoantibody frequency were also evident, with anti-p155/140, anti-MJ, and anti-Mi-2 seen more frequently in patients with JDM, anti-signal recognition particle and anti-Jo-1 in JPM, and anti-U1-RNP, PM-Scl, and other myositis-associated autoantibodies more commonly present in JCTM. Mortality was highest in patients with JCTM, whereas hospitalizations and wheelchair use were highest in JPM patients. Several demographic and clinical features were shared between juvenile and adult IIM subgroups. However, JDM and JPM patients had a lower frequency of interstitial lung disease, Raynaud phenomenon, "mechanic's hands" and carpal tunnel syndrome, and lower mortality than their adult counterparts. We conclude that juvenile myositis is a heterogeneous group of illnesses with distinct clinical subgroups, defined by varying clinical and demographic characteristics, laboratory features, and outcomes.

Phenotype

Registries

Prognosis

Humans

Logistic Models

Male

Chi-Square Distribution

Myositis - immunology

Myositis - epidemiology

Age of Onset

Statistics, Nonparametric

Autoantibodies - analysis

Female

Myositis - enzymology

Child

Myositis - classification

Details

Title: Subtitle: The clinical phenotypes of the juvenile idiopathic inflammatory myopathies
Creators: Mona Shah - From the Environmental Autoimmunity Group (MS, GM, FWM, LGR), Program of Clinical Research, National Institute of Environmental Health Sciences; Center for Information Technology (JDM), National Institutes of Health, DHHS, Bethesda, Maryland; Department of Epidemiology and Biostatistics (MS, MMR) and Division of Rheumatology, Department of Medicine(GM), George Washington University School of Medicine, Washington, DC; IWK Health Center and Dalhousie University (AMH), Halifax, Nova Scotia, Canada; and Veteran's Affairs Medical Center (INT), University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma
Gulnara Mamyrova
Ira N Targoff
Adam M Huber
James D Malley
Madeline Murguia Rice
Frederick W Miller
Lisa G Rider
Childhood Myositis Heterogeneity Collaborative Study Group
Contributors: Scott A Vogelgesang (Contributor) - University of Iowa, Internal Medicine
Resource Type: Journal article
Publication Details: Medicine (Baltimore), Vol.92(1), pp.25-41
DOI: 10.1097/MD.0b013e31827f264d
PMID: 23263716
PMCID: PMC4580479
NLM abbreviation: Medicine (Baltimore)
ISSN: 0025-7974
eISSN: 1536-5964
Grant note: R01 DK088114 / NIDDK NIH HHS Z01 ES101074 / Intramural NIH HHS Z01 ES101074-05 / Intramural NIH HHS Z99 ES999999 / Intramural NIH HHS
Language: English
Date published: 01/2013
Academic Unit: Immunology; Internal Medicine; Ophthalmology and Visual Sciences
Record Identifier: 9984094645702771

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