Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis

Rheumatology
Gulnara MamyrovaChildhood Myositis Heterogeneity Collaborative Study Group

Abstract

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. 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. 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 ...Continue Reading

References

Feb 13, 1975·The New England Journal of Medicine·A Bohan, J B Peter
Sep 6, 2005·Arthritis and Rheumatism·Chester V OddisUNKNOWN International Myositis Assessment and Clinical Studies Group
Nov 1, 2006·Arthritis and Rheumatism·Ira N TargoffUNKNOWN International Myositis Collaborative Study Group
Jun 29, 2007·The British Journal of Dermatology·P GeramiR D Sontheimer
Mar 18, 2008·Medicine·April BinghamUNKNOWN Childhood Myositis Heterogeneity Study Group
Aug 31, 2010·Rheumatology·Lisa G RiderUNKNOWN Childhood Myositis Heterogeneity Collaborative Study Group
May 10, 2013·Arthritis and Rheumatism·Mona ShahUNKNOWN Childhood Myositis Heterogeneity Collaborative Study Group
Jul 24, 2013·Medicine·Lisa G RiderUNKNOWN Childhood Myositis Heterogeneity Collaborative Study Group
Nov 5, 2014·Current Rheumatology Reports·Elizabeth E Bailey, David F Fiorentino
Oct 17, 2015·Arthritis & Rheumatology·G Esther A HabersUNKNOWN Childhood Myositis Heterogeneity Study Group
Nov 5, 2016·Pediatric Dermatology·Edward J OberleDominic O Co
Dec 13, 2017·Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG·Marie-Luise SchiffmannJan Ehrchen

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Citations

Dec 10, 2019·Current Rheumatology Reports·Jessica Neely, Susan Kim
Nov 4, 2020·Rheumatology·Gulnara MamyrovaUNKNOWN Childhood Myositis Heterogeneity Collaborative Study Group
Apr 28, 2021·Current Pediatric Reviews·Alexander K C LeungAlex H C Wong

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