Chest
Volume 113, Issue 6, June 1998, Pages 1609-1615
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Clinical Investigations: Pulmonary Hemorrhage
Isolated Pulmonary Capillaritis and Diffuse Alveolar Hemorrhage in Rheumatoid Arthritis and Mixed Connective Tissue Disease

https://doi.org/10.1378/chest.113.6.1609Get rights and content

Study objectives

To demonstrate that pulmonary capillaritis and diffuse alveolar hemorrhage (DAH) occur and are isolated to the lung and therefore not part of systemic vasculitis at the time of the DAH episode in rheumatoid arthritis (RA) and mixed connective tissue disease (MCTD).

Design

Lung biopsy specimens from patients with DAH were reviewed and those with the histologic features of pulmonary capillaritis were identified.

Setting

The patients were selected from seven Denver-area general hospitals.

Patients

Fifty-eight patients with biopsy specimen proved pulmonary capillaritis (1991 to 1997) were identified and classified according to disease. Three patients met the American Rheumatism Association criteria for RA and one patient fulfilled clinical and serologic criteria for MCTD.

Interventions

All clinical, laboratory, and radiographic data on initial presentation and at follow-up periods were extracted from the charts of the four study patients. Histologic slides were reviewed and immunofluorescent studies of lung tissue were performed.

Measurements and results

All four patients had a connective tissue disease diagnosis prior to the DAH episode. Symptoms referable to pulmonary capillaritis were of short duration (2 to 14 days) and there was no clinical or serologic evidence for an accompanying systemic vasculitis, in particular glomeronephritis. Three patients, two with RA and one with MCTD, demonstrated pulmonary immune complex deposition. Three resolved their illness following IV methylprednisilone and cyclophosphamide therapy. One RA patient died following a myocardial infarction. In the three survivors, no further episodes of DAH have occurred after a mean of 24 months (range, 10 to 48 months).

Conclusions

To our knowledge, these are the first cases of DAH due to pulmonary capillaritis documented to complicate RA and MCTD. The capillaritis was not part of a systemic vasculitis at the time of the DAH episode, but rather represented an isolated small-vessel vasculitis of the lungs in this group of patients. Immune complex deposition may be involved in the pathogenesis.

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.4,810 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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  • Cited by (0)

    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]

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