Interstitial lung disease in polymyositis and dermatomyositis. Clinical features and prognosis as correlated with histologic findings.
Open lung biopsies from 14 patients and autopsy tissue from one patient with polymyositis/dermatomyositis were reviewed in an attempt to correlate histologic features with clinical, radiographic, and prognostic variables. Three major groups based on histologic patterns were identified: bronchiolitis obliterans organizing pneumonia (BOOP), usual interstitial pneumonia (UIP), and diffuse alveolar damage (DAD). Patients with BOOP had a more favorable prognosis than did patients with UIP. Patients with DAD had a uniformly poor prognosis. One patient had a cellular interstitial pneumonia and did well. Histologic subclassification of the interstitial lung disease proved to be a better predictor of survival than did the radiographic appearance or the clinical presentation. These findings suggest that there is a broader range of histologic findings in polymyositis-dermatomyositis than is suggested in the literature and subclassification may be useful for prognosis.
Tazelaar
H D
HD
Department of Pathology, Mayo Clinic, Rochester, MN 55905.
Viggiano
R W
RW
Pickersgill
J
J
Colby
T V
TV
D000328:Adult; D000368:Aged; D001706:Biopsy; D001989:Bronchiolitis Obliterans / Q000473:pathology; D003882:Dermatomyositis / Q000150:complications*; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000150:complications*; D011014:Pneumonia / Q000473:pathology; D011379:Prognosis; D011650:Pulmonary Alveoli / Q000473:pathology; D011658:Pulmonary Fibrosis / Q000145:classification / Q000150:complications / Q000473:pathology*; D012189:Retrospective Studies