Successful Lung Transplantation in a Case of Rapidly Progressive Interstitial Lung Disease Associated with Antimelanoma Differentiation-associated Gene 5 Antibodies

Polymyositis (PM) and dermatomyositis (DM) are chronic autoimmune diseases characterized by skeletal muscle inflammation in which interstitial lung disease (ILD) is found in up to 78% of cases1. Clinically amyopathic DM, a subset of DM without objective muscle weakness, and positivity for antimelanoma differentiation-associated gene 5 autoantibodies (anti-MDA5) are known risk factors for rapidly progressive ILD (RPILD)2,3,4. We present a patient with DM, anti-MDA5 antibodies, and RPILD who successfully underwent a bilateral lung transplantation. This report was approved by the Regional Ethical Committee of Stockholm (2005/792-31/4 and 2011/1374-32). A 38-year-old white man presented with a 4-month history of uncharacteristic skin rash, mild muscle weakness, arthralgia, and exertional dyspnea. His physical examination revealed heliotrope rash, Gottron papules, periungual erythema, mechanic’s hands, small buccal mucosa erosions, fingertip ulcers, and muscle weakness. Cardiopulmonary auscultation was unremarkable. His complete blood count and muscle enzymes were normal. Erythrocyte sedimentation rate was > 90 mm/h, and C-reactive protein was 37 mg/l. He was positive for anti-Ro52 by Immunoblot, but the following were negative: anti-Mi2, anti-SRP, anti-Jo1, anti-PL7, anti-PL12, anti-dsDNA, anti-Sm, anti-Scl-70, anti-Scl-100, anti-SS/B, anti-RNP, rheumatoid factor, antinuclear antibody, and antineutrophil cytoplasmic antibodies. Muscle histopathology showed fiber size variation. A chest computer tomography (CT) showed unspecific basal reticular infiltrates. The patient was started on … Address correspondence to Dr. I.E. Lundberg, Rheumatology Unit, Karolinska University Hospital, Stockholm 17176, Sweden. E-mail: Ingrid.lundberg{at}ki.se.