Interstitial lung disease in polymyositis and dermatomyositis

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Abstract
Objectives: To assess prevalence, characteristics, and long‐term outcome of interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM). To determine predictive variables of ILD course in PM/DM, and to define both clinical and biochemical features associated with ILD onset in PM/DM.Methods: The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed.Results: Thirty‐six PM/DM patients (23.1%) developed ILD. We observed that 19.4% of patients with ILD had resolution of pulmonary disorders, whereas 25% experienced ILD deterioration. Morbidity and mortality rates were as high as 13.9% and 36.4%, respectively, in PM/DM patients with ILD. Parameters of PM/DM that related to ILD poor outcome were identified as follows: Hamman‐Rich–like pattern, initial diffusing capacity of carbon monoxide <45%, neutrophil alveolitis, and histologic usual interstitial pneumonia. Additionally, for the group with ILD, polyarthritis, higher values of erythrocyte sedimentation rate and C‐reactive protein, presence of anti–Jo‐1 antibody, and characteristic microangiopathy were significantly more frequent.Conclusion: Our series underlines the high frequency of ILD in PM/DM patients, resulting in increased morbidity and mortality rates. It also indicates that PM/DM patients should routinely be screened for ILD, even those patients without anti–Jo‐1 antibody, because 69% of our ILD patients were seronegative for the anti–Jo‐1 antibody. Our findings further suggest that PM/DM patients presenting with factors predictive of ILD poor outcome may require more aggressive therapy.