Autoimmune inflammatory myositis which commonly has pulmonary involvement with interstitial lung disease (ILD).

Constellation of clinical findings including hand deformity (mechanic's hands), Raynaud's phenomenon and polyarthritis.

The pattern of ILD can be NSIP, organizing pneumonia or UIP, with a basal and peripheral distribution, which responds to steroid therapy. 

Patient's can develop pulmonary hypertension.