Journal article
Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis
Rheumatology (Oxford, England), Vol.57(11), pp.1956-1963
11/01/2018
DOI: 10.1093/rheumatology/key190
PMCID: PMC6199536
PMID: 30016492
Abstract
Abstract
Objective
We examined features of clinically amyopathic JDM (CAJDM), in which patients have characteristic rashes with little to no evidence of muscle involvement, to determine whether this is a distinct phenotype from JDM.
Methods
Demographic, clinical, laboratory and treatment data from 12 (9 hypomyopathic, 3 amyopathic) patients meeting modified Sontheimer criteria for CAJDM and from 60 matched JDM patients meeting Bohan and Peter criteria were examined. Differences were evaluated by Fisher's exact and Mann-Whitney tests, random forests and logistic regression analysis.
Results
Nine (75%) CAJDM patients had anti-p155/140 (transcriptional intermediary factor 1), one (8.3%) anti-melanoma differentiation-associated gene 5 autoantibodies and two (16.7%) were myositis autoantibody negative. CAJDM patients were younger at diagnosis and frequently had mild disease at onset. CAJDM patients had less frequent myalgias, arthritis, contractures, calcinosis, dysphagia, abdominal pain and fatigue. The muscle, skeletal and overall clinical scores were lower in CAJDM. Serum muscle enzymes were less frequently increased in CAJDM, and peak values were lower. CAJDM patients received fewer medications compared with JDM patients. Only 50% of CAJDM patients received oral prednisone, but the maximum dose and treatment duration did not differ from JDM. At a median follow-up of 2.9 years, CAJDM patients had no documented functional disability, and none developed weakness, calcinosis, interstitial lung disease or lipodystrophy. Multivariable modelling revealed a lower skeletal score and less frequent myalgias as the most important factors in distinguishing CAJDM from JDM.
Conclusion
CAJDM may be distinguished from JDM, in that they often have p155/140 (transcriptional intermediary factor 1) autoantibodies, have fewer systemic manifestations and receive less therapy.
Details
- Title: Subtitle
- Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis
- Creators
- Gulnara Mamyrova - George Washington UniversityTakayuki Kishi - National Institute of Environmental Health SciencesIra N Targoff - University of Oklahoma Health Sciences CenterAlison Ehrlich - George Washington UniversityRodolfo V Curiel - George Washington UniversityLisa G Rider - George Washington UniversityChildhood Myositis Heterogeneity Collaborative Study Group
- Contributors
- Scott A Vogelgesang (Contributor) - University of Iowa, Internal Medicine
- Resource Type
- Journal article
- Publication Details
- Rheumatology (Oxford, England), Vol.57(11), pp.1956-1963
- Publisher
- Oxford University Press
- DOI
- 10.1093/rheumatology/key190
- PMID
- 30016492
- PMCID
- PMC6199536
- ISSN
- 1462-0324
- eISSN
- 1462-0332
- Grant note
- ES101074; ES1010081 / National Institute of Environmental Health Sciences (10.13039/100000066)
- Language
- English
- Date published
- 11/01/2018
- Academic Unit
- Immunology; Internal Medicine; Ophthalmology and Visual Sciences
- Record Identifier
- 9984359817802771
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