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Diffuse panbronchiolitis

Author
Talmadge E King, Jr, MD
Section Editor
Kevin R Flaherty, MD, MS
Deputy Editor
Helen Hollingsworth, MD

INTRODUCTION

Diffuse panbronchiolitis (DPB) is a rare clinicopathologic syndrome characterized by bronchiolitis and chronic sinusitis [1,2]. In the disease name, "diffuse" refers to the distribution of the lesions throughout both lungs and "pan" refers to the pathologic finding that the inflammation involves all layers of the respiratory bronchioles [1].

The predisposing factors, clinical manifestations, evaluation, diagnosis, and treatment of diffuse panbronchiolitis will be discussed here. The clinical features, diagnosis, and management of other types of bronchiolitis in adults and children are reviewed separately. (See "Bronchiolitis in adults" and "Bronchiolitis in infants and children: Clinical features and diagnosis" and "Bronchiolitis in infants and children: Treatment; outcome; and prevention".)

PATHOGENESIS

The exact pathogenesis of DPB is not known. A variety of genetic, environmental, and systemic factors appear to contribute.

Genetic factors — Genetic factors are suspected to contribute to the pathogenesis of DPB based on the observation of familial cases and the association of specific human leukocyte antigen (HLA) haplotypes with the disease. In addition to familial cases noted in Japan, it is also common to identify chronic sinusitis without bronchiolitis in first-degree relatives of afflicted individuals [3-5].

Specific HLA haplotypes, such as HLA-B54 in Japan and HLA-A11 in Korea, are associated with the development of DPB [5,6]. In one report, for example, HLA-B54 was identified in 63 percent of Japanese patients with DPB versus 11 percent of normal Japanese controls (relative risk 13.3) [5]. This HLA haplotype has been reported almost exclusively in natives of Japan, China, and Korea, where it is also associated with rheumatoid arthritis and silicosis.

                          

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Literature review current through: Nov 2016. | This topic last updated: Mon Jul 11 00:00:00 GMT+00:00 2016.
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