PLoS ONE (Jan 2014)

Prognostic factors for myositis-associated interstitial lung disease.

  • Tomoyuki Fujisawa,
  • Hironao Hozumi,
  • Masato Kono,
  • Noriyuki Enomoto,
  • Dai Hashimoto,
  • Yutaro Nakamura,
  • Naoki Inui,
  • Koshi Yokomura,
  • Naoki Koshimizu,
  • Mikio Toyoshima,
  • Toshihiro Shirai,
  • Kazumasa Yasuda,
  • Hiroshi Hayakawa,
  • Takafumi Suda

DOI
https://doi.org/10.1371/journal.pone.0098824
Journal volume & issue
Vol. 9, no. 6
p. e98824

Abstract

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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.