LAUSR

OBJECTIVES To describe the 8-year longitudinal study and long-term prognosis of a large inception cohort of anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive (MDA5+) dermatomyositis (DM)-interstitial lung disease (ILD) patients. METHODS 216 patients diagnosed with MDA5+ DM-ILD were enrolled and followed up to analyze long-term survival rate. Demographic and clinical variables were collected at baseline and each temporal end point. 70 patients who survived the first year were analyzed for the long-term serological and respiratory outcomes. RESULTS 85 patients (39.3%) died during the follow-up period up to 96 months, with 89% of the deaths occurring in the first year after diagnosis. Long-term outcome was reported in 70 patients. Serological markers including anti-MDA5 antibody showed significant improvement with time. Radiographic findings and pulmonary function also improved notably in the follow-up period, especially in rapidly progressive ILD group, as measured by high-resolution computed tomography imaging scores, the estimated forced vital capacity, estimated diffusing capacity of lung carbon monoxide and dyspnea scores. Early application of anti-fibrosis therapy helped to improve long-term pulmonary function. CONCLUSIONS MDA5+ DM-ILD patients had a high mortality rate despite aggressive treatment. Patients who survived the first year usually showed a significant improvement in serological markers and pulmonary function during the long-term follow-up.