Long-term outcome of 53 patients with anti-Jo-1 antibody-positive interstitial lung disease

Background: Anti-Jo-1 antibody is often identified in patients with polymyositis/dermatomyositis (PM/DM). Patients with interstitial lung disease with anti-Jo-1 antibody (Jo-1-ILD) but without PM/DM tend to be recognized as idiopathic interstitial pneumonias (IIPs) and their long-term prognosis is not well understood. Methods: 53 patients diagnosed as Jo-1-ILD in our hospital from June 1990 to June 2016 were retrospectively reviewed. We compared the survival between patients with PM/DM (PM/DM-ILD) and without clinically recognized PM/DM (non-PM/DM-ILD) and evaluated clinical, radiological and histological features. Results: Non-PM/DM-ILD had 29 patients who were older than patients in PM/DM-ILD(62.4 years vs. 53.7 years, p Conclusions: Non-PM/DM-ILD, Jo-1-ILD without PM/DM had as good long-term outcome as PM/DM-ILD did. Jo-1-ILD should be categorized differently from IIPs.