Analysis of HLA Antigens in Patients with Diffuse Panbronchiolitis

Abstract

A new clinicopathologic entity, diffuse panbronchiolitis, was recently reported in Japan. This disorder is characterized by recurrent sinopulmonary infection and respiratory bronchiolitis. We identified cases of familial diffuse panbronchiolitis and found that many siblings of the patients has paranasal sinusitis. Because of this, we performed HLA anaysis of the patients with diffuse panbronchiolitis. In HLA Class I and Class II antigens, Bw54 significantly increased (frequency: 63.2%; RR = 113.30; corrected p value < 1.08 .times. 10-10), whereas Cw1 and MC1 (a new HA-DR-related antigen) slightly increased. These incresease might be explained by the fact that these two antigens, Cw1 and MC1, formed a haplotype with Bw54, which suggests that one or some of the genes controlling the susceptibility and/or immune responsiveness of diffuse panbronchiolitis might be located near HLA loci. In addition, Bw54, or its related haplotypes, exists only in Japanese, Chinese, and Korean populations. This may explain why a similar disease had not been reported in the English literature.