title: Prognostic factors for myositis-associated interstitial lung disease.
abstract: BACKGROUND
  Interstitial lung disease (ILD) is a common manifestation of polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM); however, little is known about the factors influencing the prognosis for PM/DM/CADM-associated ILD. (PM/DM/CADM-ILD). The aim of the present study is to assess prognostic factors for PM/DM/CADM-ILD.
  
  
  METHODS
  The clinical features and survival of 114 consecutive patients diagnosed with PM/DM/CADM-ILD (39 men and 75 women; median age, 56 years) were analyzed retrospectively.
  
  
  RESULTS
  The study group included 30 PM-associated ILD, 41 DM-associated ILD, and 43 CADM-associated ILD cases. The clinical presentation of ILD was acute/subacute form in 59 patients (51.8%) and chronic form in 55 patients (48.2%). The major pulmonary symptoms were dyspnea, cough, and fever. High-resolution computed tomography frequently revealed ground-glass opacities, traction bronchiectasis, and consolidation. Most of the patients were treated with corticosteroids or corticosteroids in combination with immunosuppressive agents. The all-cause mortality was 27.2%. Acute/subacute form, % forced vital capacity (FVC), age, % of neutrophils in bronchoalveolar lavage (BAL) fluid, and a diagnosis of CADM (vs. PM) were significantly associated with poor outcome in univariate Cox proportional hazards models. Multivariate Cox proportional hazards analysis validated acute/subacute ILD, %FVC, age, and diagnosis of CADM (vs. PM) as significant predictors of overall mortality. Patients with acute/subacute ILD had a much lower survival rate than those with the chronic form (p<0.001). Patients with CADM-ILD had a lower survival rate than those with PM-ILD (p = 0.034).
  
  
  CONCLUSIONS
  Acute/subacute form, older age, lower level of FVC and diagnosis of CADM predict poor outcome in PM/DM/CADM-ILD.
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Fujisawa
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Hironao
authors.initials: H
authors.lastname: Hozumi
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Masato
authors.initials: M
authors.lastname: Kono
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Enomoto
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Dai
authors.initials: D
authors.lastname: Hashimoto
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Yutaro
authors.initials: Y
authors.lastname: Nakamura
authors.affiliation: Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Inui
authors.affiliation: Department of Respiratory Medicine, Seirei Mikatahara General Hospital, Hamamatsu, Japan.
authors.forename: Koshi
authors.initials: K
authors.lastname: Yokomura
authors.affiliation: Department of Respiratory Medicine, Fujieda Municipal General Hospital, Fujieda, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Koshimizu
authors.affiliation: Department of Respiratory Medicine, Hamamatsu Rosai Hospital, Hamamatsu, Japan.
authors.forename: Mikio
authors.initials: M
authors.lastname: Toyoshima
authors.affiliation: Department of Respiratory Medicine, Shizuoka General Hospital, Shizuoka, Japan.
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Shirai
authors.affiliation: Department of Respiratory Medicine, Iwata City Hospital, Iwata, Japan.
authors.forename: Kazumasa
authors.initials: K
authors.lastname: Yasuda
authors.affiliation: Department of Respiratory Medicine, Tenryu Hospital, National Hospital Organization, Hamamatsu, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hayakawa
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Suda
mesh_terms: D000368:Aged; D019411:Clinical Laboratory Techniques; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000150:complications*; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed