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Semin Respir Crit Care Med 1999; 20(2): 149-168
DOI: 10.1055/s-2007-1009456
Copyright © 1999 by Thieme Medical Publishers, Inc.

Bronchiolar Complications of Connective Tissue Disorders

Joseph P. Lynch*  III , John Belperio* , Andrew Flint† , Fernando Martinez*
  • *Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, and the
  • †Department of Pathology, University of Michigan Medical Center, Ann Arbor, Michigan
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Publication History

Publication Date:
20 March 2008 (online)

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Abstract

A spectrum of inflammatory and fibrotic disorders involving the small airways (i.e., respiratory and terminal bronchioles) may complicate connective tissue disorders (CTDs). Obliterative (constrictive) bronchiolitis (OB) is a well-recognized, albeit rare, complication of rheumatoid arthritis, and occasional cases have been described in other CTDs. Bronchiolitis obliterans with organizing pneumonia (BOOP), also termed cryptogenic organizing pneumonia, is a rare complication of CTDs. Clinical, radiographic, histological features and prognosis differ markedly between these entities. Clinical features are nonspecific, and the diagnosis (particularly OB) may be difficult. High resolution thin section computed tomographic (HRCT) scans are useful in detecting bronchiolar pathology, even when symptoms are minimal or absent. Surgical (open or thoracoscopic) lung biopsies can substantiate the diagnosis, but in some cases, the diagnosis can be affirmed less aggressively by appropriate imaging studies (e.g., HRCT) and transbronchial lung biopsies. Corticosteroids are highly efficacious for BOOP, but therapeutic options for OB are disappointing.

Key Words:

airflow obstruction - bronchiolitis - obliterative bronchiolitis - constrictive bronchiolitis - bronchiolitis obliterans organizing pneumonia - cryptogenic organizing pneumonia - connective tissue diseases - rheumatoid arthritis

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