Chest
Clinical Investigations: Pulmonary HemorrhageIsolated Pulmonary Capillaritis and Diffuse Alveolar Hemorrhage in Rheumatoid Arthritis and Mixed Connective Tissue Disease
Section snippets
MATERIALS AND METHODS
During the period from 1991 to 1997, 58 patients with DAH and pulmonary capillaritis were identified at seven Denver-area hospitals. During this period, the histology of all DAH patients who underwent lung biopsy was reviewed. Twenty-five of these patients were subjects of prior publications.4,8–10 The diagnosis of pulmonary capillaritis was established by open or thoracoscopic lung biopsy specimens in 56 patients and by transbronchial biopsy specimens in two. In the four subjects who form the
Patient Characteristics and Presenting Manifestations
Three patients fulfilled the criteria for RA and one for MCTD (Tables 2 and 3). Their age range was wide and all had their primary diagnosis established prior to the episode of DAH (duration, 2 to 40 years). Only one subject with RA was actively smoking. Dyspnea and cough were present in all subjects, and hemoptysis was reported by three. Symptoms were ongoing from 2 to 14 days before evaluation. Only one patient was febrile. No patient had accompanying systemic manifestations, including a
DISCUSSION
We have described three patients with previously diagnosed RA and one with MCTD who developed DAH due to biopsy specimen proved necrotizing pulmonary capillaritis. To our knowledge, these are the first documented cases of pulmonary capillaritis accompanying these disorders. Furthermore, this small-vessel vasculitis was localized to the lung, serum ANCA was absent, and there were no other clinical features to suggest an accompanying acute systemic vasculitis at the time of the DAH episode. There
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Supported by National Heart, Lung, and Blood SCOR Institute Grant HL27353-04. Also supported by National Heart, Lung and Blood Institute Training Grant HL-07085 (Dr. Chan).
Reprint requests: Marvin I. Schwarz, MD, FCCP, The James C. Campbell Professor of Pulmonary Medicine, 4200 E Ninth Ave, C272, Denver, CO 80262; email: [email protected]