Clinical characteristics of anti-Jo-1-positive interstitial lung disease

Anti-Jo-1-syndrome is the most common form of antisynthetase syndrome, which often presents with interstitial lung disease (ILD) and polymyositis/dermatomyositis(PM/DM). Little is understood how the presence of PM/DM affects the clinical presentation of ILD patients with anti-Jo-1-antibody (Jo-1-ILD).We aim to characterize Jo-1-ILD by comparing patients with PM/DM (PM/DM-ILD) and without PM/DM (non-PM/DM-ILD).In this retrospective review, 49 Jo-1-ILD patients diagnosed at our institution from January 2005 to June 2018 were included. Clinical, radiologic, and histologic features of 20 PM/DM-ILD and 29 non-PM/DM-ILD patients were compared. PM/DM-ILD patients were younger at ILD diagnosis than non-PM/DM-ILD (51.0 years vs. 64.5 years, p<0.001). The presence of PM/DM was positively correlated with anti-Ro antibody status (R=0.372, p=0.009). Higher proportions of PM/DM-ILD were treated with glucocorticoids (95.0% vs. 44.8%, p<0.001) and/or immunosuppressants (65.0% vs. 17.2%, p<0.001) than non-PM/DM-ILD. Radiologic evaluation showed a predominance of nonspecific interstitial pneumonia (NSIP) pattern in both groups. Histologic evaluation of 20 patients (41.0%) shows a heterogenenous pathology. Similar proportions of PM/DM-ILD and non-PM/DM-ILD received lung transplantation (20% vs. 13.7%, p=0.56). Among 11 deaths (22.4%) from both groups, 2 patients from each group died of acute exacerbation of ILD. 10-year survival rates of PM/DM-ILD and non-PM/DM-ILD were 64.3% and 58.2%...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research