Fatal interstitial pulmonary fibrosis in anti-Jo-1-negative amyopathic dermatomyositis

Amyopathic dermatomyositis (DM) describes a subpopulation with the cutaneous eruption of DM, but without muscle involvement. Interstitial pulmonary fibrosis is a recognized complication of DM, often correlated with antisynthetase enzymes, such as anti-Jo-1. We describe a case of fatal IPF in a patient with anti-Jo-1 antibody-negative amyopathic DM.