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.
High
Whitney A
WA
Department of Dermatology, University of Texas Sothwestern Medical Center, Dallas, Texas 75390-9190, USA.
Cohen
Jack B
JB
Murphy
Bettina A
BA
Costner
Melissa I
MI
D000974:Antibodies, Antinuclear; D003882:Dermatomyositis / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D017809:Fatal Outcome; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D011658:Pulmonary Fibrosis / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D012131:Respiratory Insufficiency / Q000209:etiology
D000974:Antibodies, Antinuclear; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; C046750:Jo-1 antibody; D011241:Prednisone; D008727:Methotrexate