title: Polymyositis associated with severe interstitial lung disease: remission after three doses of IV immunoglobulin.
abstract: Interstitial lung disease associated with polymyositis/dermatomyositis (ILD-PM/DM) often confers a poor prognosis, and optimal treatment of this condition is not well defined. This report describes a 63-year-old man with severe ILD-PM/DM who presented with 5 months of progressive dyspnea and weakness. He had an initial carbon-monoxide-diffusing capacity of 35% predicted and a creatine kinase level of 2,112 U/L. After three monthly doses of immunoglobulin at 2 gm/kg IV, he has sustained clinical remission for > 2 years. IV immunoglobulin has not previously been studied as a first-line agent for rheumatologic diseases, and it is currently used as a salvage therapy. However, if IV immunoglobulin is capable of inducing sustained remission after brief use as a treatment, as demonstrated in this patient, especially in the setting of significant pulmonary involvement, then it merits further consideration for investigation as a first-line therapeutic agent.
authors.affiliation: Division of Rheumatology, University of Washington Medical Center, Seattle, WA. Electronic address: bakewell@uw.edu.
authors.forename: Catherine J
authors.initials: CJ
authors.lastname: Bakewell
authors.affiliation: Division of Pulmonary and Critical Care Medicine, University of Washington Medical Center, Seattle, WA.
authors.forename: Ganesh
authors.initials: G
authors.lastname: Raghu
mesh_terms: D003882:Dermatomyositis / Q000150:complications* / Q000188:drug therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged
chemical_list: D016756:Immunoglobulins, Intravenous