Anti-CADM-140 Antibody-positive Juvenile Dermatomyositis with Rapidly Progressive Interstitial Lung Disease and Cardiac Involvement

Abstract

To the Editor: Extramusculocutaneous manifestations in juvenile dermatomyositis (JDM) may lead to life-threatening consequences. Interstitial lung disease (ILD) has been reported as one such serious complication in JDM1,2,3,4; how ever, cardiac involvement in JDM is a rare complication and is seldom reported5,6. Recently, anti-CADM-140 autoantibody was discovered in amyopathic dermatomyositis and was associated with rapidly progressive ILD7,8. We describe a fatal case of JDM complicated by ILD and cardiac involvement in which serum preserved at admission was shown to contain anti-CADM-140 antibody. A 9-year-old boy was admitted to our hospital with a 4-month history of low-grade fever and erythematous rashes on his face, hands, elbows, and knees. He was developmentally delayed from an unknown cause. He could not describe muscle weakness or tenderness but showed claudication indicating lower-limb muscle weakness. He had Gottron’s papules but no heliotrope rash. Cardiac sounds revealed a gallop rhythm and he had fine crackles over both lung fields. His erythrocyte sedimentation rate was 38 mm/h, white cell count 1800/μl, … Address correspondence to Dr. Nagai; E-mail: nkazu{at}sapmed.ac.jp