Predictors of Poor Outcome of Anti‐MDA5‐Associated Rapidly Progressive Interstitial Lung Disease in a Chinese Cohort with Dermatomyositis

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Objective . Antimelanoma differentiation‐associated protein 5 (anti‐MDA5) autoantibody has been reported in dermatomyositis (DM) to be associated with rapidly progressive interstitial lung disease (RP‐ILD). Our study is aimed at determining the clinical characteristics and prognostic factors underpinning anti‐MDA5‐associated RP‐ILD. Methods . Patients with anti‐MDA5‐associated DM (aMDA5‐DM) were identified at the Peking University People’s Hospital. The presence of anti‐MDA5 antibody was determined by immunoblotting. Kaplan‐Meier, chi‐square test, univariate, and multivariate data analyses were used. Results . Out of 213 patients with DM and clinically amyopathic dermatomyositis (CADM), 20.7% (44/213) of patients were identified as aMDA5‐DM. Amongst the aMDA5‐DM patients, 63.6% (28/44) were identified as having anti‐MDA5‐associated RP‐ILD. During the follow‐up, 32.1% (9/28) of patients with anti‐MDA5‐associated RP‐ILD died of respiratory failure. We identified older age and periungual erythema as two independent risk factors for RP‐ILD mortality. Age ≥ 57 years at disease onset was significantly associated with poor survival ( P = 0.02) in patients with anti‐MDA5‐associated RP‐ILD, while patients with periungual erythema had a better survival rate than those without periungual erythema ( P < 0.05). Conclusions . Anti‐MDA5‐associated RP‐ILD is significantly associated with poor survival rates in DM/CADM patients. More effective intervention should be administered to anti‐MDA5‐associated RP‐ILD patients, especially to senior patients and those without periungual erythema.