Microscopic polyangiitis presenting as a “pulmonary-muscle” syndrome: Is subclinical alveolar hemorrhage the mechanism of pulmonary fibrosis?
Article first published online: 25 MAY 2007
Copyright © 2007 by the American College of Rheumatology
Arthritis & Rheumatism
Volume 56, Issue 6, pages 2065–2071, June 2007
How to Cite
Birnbaum, J., Danoff, S., Askin, F. B. and Stone, J. H. (2007), Microscopic polyangiitis presenting as a “pulmonary-muscle” syndrome: Is subclinical alveolar hemorrhage the mechanism of pulmonary fibrosis?. Arthritis & Rheumatism, 56: 2065–2071. doi: 10.1002/art.22633
- Issue published online: 25 MAY 2007
- Article first published online: 25 MAY 2007
- Manuscript Accepted: 20 FEB 2007
- Manuscript Received: 1 SEP 2006
Microscopic polyangiitis (MPA) may present with a syndrome that resembles idiopathic pulmonary fibrosis (IPF). We describe an MPA patient with the clinical presentation of a “pulmonary-muscle” syndrome in which interstitial lung disease antedated the onset of myopathy. Identification of vasculitis on muscle biopsy was instrumental in recognizing clinical, radiographic, and histopathologic features that were more characteristic of MPA than of IPF. Institution of glucocorticoid and cyclophosphamide therapy led to the induction of a complete remission. The histologic findings in this case implicate subclinical episodes of alveolar hemorrhage as the mechanism of interstitial lung disease in MPA.