PReS-FINAL-2130: Antibodies to MDA5 correlate with a distinct phenotype in children with juvenile dermatomyositis, including higher risk of lung involvement and ulcerative skin disease

E Moraitis,N Mchugh,H Gunawardena,S Tansley,K Arnold,C Owens,Lr Wedderburn,Ze Betteridge,Ts Jacques

doi:10.1186/1546-0096-11-s2-p142

Abstract

Myositis specific autoantibodies (MSA), exclusively found in patients with Idiopathic Inflammatory Myopathies can be detected in approximately 60% of children with JDM. Anti-MDA5 antibodies, a subgroup of MSA, appear to be associated with clinically amyopathic myositis, rapidly progressive interstitial lung disease (RP-ILD) and a poor prognosis in adult East Asian dermatomyositis patients. Small studies in Japanese children with JDM have suggested similar disease phenotype. This contrasts dramatically with findings in predominantly Caucasian US adult populations where anti-MDA5 has been associated with a distinct cutaneous phenotype and no association with RP-ILD has been found.

Highlights

Pediatric Rheumatology European Society (PReS)-FINAL-2130: Antibodies to MDA5 correlate with a distinct phenotype in children with juvenile dermatomyositis, including higher risk of lung involvement and ulcerative skin disease
Myositis specific autoantibodies (MSA), exclusively found in patients with Idiopathic Inflammatory Myopathies can be detected in approximately 60% of children with JDM
Anti-MDA5 antibodies were identified in 7.4% of JDM patients and were associated with a distinct clinical phenotype including skin ulceration (p = 0.025), oral ulceration (p = 0.013), arthritis (p = 0.002) and milder muscle disease both clinically (as determined by a higher lowest ever Childhood Myositis Activity Score (p = 0.024)) and histologically