title: [Outcome in patients with interstitial lung disease and polymyositis-dermatomyositis--a subgroup with poor prognosis].
abstract: To determine the role of interstitial lung disease (ILD) in the outcome of polymyositis-dermatomyositis (PM/DM), we studied 41 patients who had radiographic evidence of ILD among 51 patients with PM/DM. The patients were divided into 3 groups according to therapy: progressive, no change, or improved. Those classified as progressive had significantly higher rates of skin and lung symptoms, milder muscle symptoms, and lower levels of muscle enzymes than the other 2 groups, and none was positive for the anti-Jo-1 autoantibody. Nine patients with DM died of respiratory failure; they had low serum creatine kinase levels, no anti-Jo-1 antibodies, and a rapidly progressive course before death. Autopsy findings were obtained in 7 patients, and all had diffuse alveolar damage as the main pulmonary histological finding. A subgroup of patients with PM/DM-ILD have a poor prognosis, and ILD in PM/DM is an important prognostic factor.
authors.affiliation: Department of Internal Medicine & Physical Therapy, University of Tokyo, School of Medicine, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Takizawa
authors.forename: N
authors.initials: N
authors.lastname: Suzuki
authors.forename: T
authors.initials: T
authors.lastname: Yanagawa
authors.forename: H
authors.initials: H
authors.lastname: Okazaki
authors.forename: M
authors.initials: M
authors.lastname: Satoh
authors.forename: N
authors.initials: N
authors.lastname: Akiyama
authors.forename: T
authors.initials: T
authors.lastname: Kohyama
authors.forename: S
authors.initials: S
authors.lastname: Kawasaki
authors.forename: K
authors.initials: K
authors.lastname: Ito
authors.forename: T
authors.initials: T
authors.lastname: Oka
mesh_terms: D003882:Dermatomyositis / Q000150:complications*; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000150:complications*; D011379:Prognosis