Interstitial lung disease-associated antisynthetase syndrome

Abstract

Interstitial lung disease (ILD) is one of the major extramuscular manifestations of polymyositis (PM) and dermatomyositis (DM). Presentation of PM and DM with ILD is not uncommon, but its clinical and radiological features can be similar to other conditions and can be challenging to diagnose. The presence of anti-aminoacyl tRNA synthetase antibodies in the presence of ILD is diagnostic of antisynthetase syndrome. A delayed diagnosis can be associated with the progression of pulmonary involvement and potentially increased morbidity. Timely diagnosis leads to appropriable lifesaving treatment. We report on a patient with chronic respiratory symptoms who had positive anti-Jo-1 antibodies and presented in respiratory failure, requiring non-invasive ventilation.