Interstitial lung disease in polymyositis and dermatomyositis
Article first published online: 12 DEC 2002
Copyright © 2002 by the American College of Rheumatology
Arthritis Care & Research
Volume 47, Issue 6, pages 614–622, 15 December 2002
How to Cite
Marie, I., Hachulla, E., Chérin, P., Dominique, S., Hatron, P.-Y., Hellot, M.-F., Devulder, B., Herson, S., Levesque, H. and Courtois, H. (2002), Interstitial lung disease in polymyositis and dermatomyositis. Arthritis & Rheumatism, 47: 614–622. doi: 10.1002/art.10794
- Issue published online: 12 DEC 2002
- Article first published online: 12 DEC 2002
- Manuscript Accepted: 10 MAR 2002
- Manuscript Received: 8 NOV 2001
- Interstitial lung disease;
- Anti–Jo-1 antibody
To assess prevalence, characteristics, and long-term outcome of interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM). To determine predictive variables of ILD course in PM/DM, and to define both clinical and biochemical features associated with ILD onset in PM/DM.
The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed.
Thirty-six PM/DM patients (23.1%) developed ILD. We observed that 19.4% of patients with ILD had resolution of pulmonary disorders, whereas 25% experienced ILD deterioration. Morbidity and mortality rates were as high as 13.9% and 36.4%, respectively, in PM/DM patients with ILD. Parameters of PM/DM that related to ILD poor outcome were identified as follows: Hamman-Rich–like pattern, initial diffusing capacity of carbon monoxide <45%, neutrophil alveolitis, and histologic usual interstitial pneumonia. Additionally, for the group with ILD, polyarthritis, higher values of erythrocyte sedimentation rate and C-reactive protein, presence of anti–Jo-1 antibody, and characteristic microangiopathy were significantly more frequent.
Our series underlines the high frequency of ILD in PM/DM patients, resulting in increased morbidity and mortality rates. It also indicates that PM/DM patients should routinely be screened for ILD, even those patients without anti–Jo-1 antibody, because 69% of our ILD patients were seronegative for the anti–Jo-1 antibody. Our findings further suggest that PM/DM patients presenting with factors predictive of ILD poor outcome may require more aggressive therapy.