title: Interstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and radiology.
abstract: OBJECTIVE
  To estimate predictors and long-term outcome of interstitial lung disease (ILD) in patients with polymyositis (PM) and dermatomyositis (DM).
  
  
  METHODS
  We conducted a prospective study in which newly diagnosed PM/DM patients, regardless of clinical symptoms of pulmonary disease, were investigated with repeated chest radiography, high-resolution computed tomography (HRCT) of the lungs, and pulmonary function test (PFT). Clinical, radiologic, and lung function outcome was based on the last followup results.
  
  
  RESULTS
  Twenty-three patients with a mean followup period of 35 months were included. Findings on radiographic examination and/or PFT compatible with ILD were recorded in 18 patients (78%). Patients with ILD had lower lung function, higher radiologic scores, and higher creatine kinase values than those without ILD. All patients were treated with high-dose glucocorticoids and other immunosuppressive agents. Two patients died due to ILD, both with active myositis. During the followup, total lung capacity (TLC) improved in 33%, remained stable in 39%, and deteriorated in 28%. Changes in TLC correlated only partially with HRCT findings, which persisted even after normalizing for lung function.
  
  
  CONCLUSIONS
  ILD associated with PM/DM is in most cases mild, chronic, and has a nonprogressive course during immunosuppressive treatment. PFT can be normalized during treatment with immunosuppressive therapy, even if radiologic signs of ILD persist. The course of ILD could not be predicted on the first examination. Therefore, myositis patients with ILD need careful evaluation of clinical features as well as PFT and radiologic features during followup.
authors.affiliation: Karolinska University Hospital, Solna, Sweden. Maryam.Fathi@Karolinska.se
authors.forename: Maryam
authors.initials: M
authors.lastname: Fathi
authors.forename: Jenny
authors.initials: J
authors.lastname: Vikgren
authors.forename: Marianne
authors.initials: M
authors.lastname: Boijsen
authors.forename: Ulf
authors.initials: U
authors.lastname: Tylen
authors.forename: Lennart
authors.initials: L
authors.lastname: Jorfeldt
authors.forename: Göran
authors.initials: G
authors.lastname: Tornling
authors.forename: Ingrid E
authors.initials: IE
authors.lastname: Lundberg
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003882:Dermatomyositis / Q000150:complications*; D005260:Female; D005544:Forecasting; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000150:complications*; D011859:Radiography; D012129:Respiratory Function Tests