Polymyositis/Dermatomyositis

You have full text access to this OnlineOpen article

Interstitial lung disease in polymyositis and dermatomyositis: Longitudinal evaluation by pulmonary function and radiology

  1. Maryam Fathi1,4,*,
  2. Jenny Vikgren2,
  3. Marianne Boijsen2,
  4. Ulf Tylen2,
  5. Lennart Jorfeldt3,
  6. Göran Tornling1,4,
  7. Ingrid E. Lundberg1,4

Article first published online: 25 APR 2008

DOI: 10.1002/art.23571

Issue

Arthritis Care & Research

Arthritis Care & Research

Volume 59, Issue 5, pages 677–685, 15 May 2008

Additional Information

How to Cite

Fathi, M., Vikgren, J., Boijsen, M., Tylen, U., Jorfeldt, L., Tornling, G. and Lundberg, I. E. (2008), Interstitial lung disease in polymyositis and dermatomyositis: Longitudinal evaluation by pulmonary function and radiology. Arthritis Care & Research, 59: 677–685. doi: 10.1002/art.23571

Author Information

  1. 1

    Karolinska University Hospital, Solna, Sweden

  2. 2

    The Sahlgrenska Academy of Göteborg University, Sahlgrenska University Hospital, Göteborg, Sweden

  3. 3

    Karolinska Institutet, Stockholm, Sweden

  4. 4

    Karolinska Institutet, Stockholm, Sweden

*Department of Respiratory Medicine and Allergy, Karolinska University Hospital, SE-171 76 Stockholm, Sweden

Publication History

  1. Issue published online: 25 APR 2008
  2. Article first published online: 25 APR 2008
  3. Manuscript Accepted: 2 NOV 2007
  4. Manuscript Received: 4 AUG 2006

Funded by

  • The Swedish Rheumatism Association
  • King Gustaf V's 80-Year Foundation
  • The Swedish Research Council. Grant Number: 2002-74X-14045-02A
  • Professor Nanna Svartz Foundation
  • Karolinska Institutet Foundation
  • Swedish Heart Lung Foundation

SEARCH

SEARCH BY CITATION

ARTICLE TOOLS

View Full Article (HTML) Get PDF (110K)

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.

Conclusion

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.

View Full Article (HTML) Get PDF (110K)