CLINICAL PROFILES OF CHINESE PATIENTS WITH DIFFUSE PANBRONCHIOLITIS

Background-Diffuse panbronchiolitis (DPB), characterised by progressiv e sino-bronchial sepsis, is well characterised in. Japanese subjects b ur not in other ethnic groups. The experience with DPB in seven Chines e patients is described and the clinical profiles compared with those of Japanese subjects. Methods-Seven Chinese patients (three women; mea n (SD) age 48(18.6) years, all never smokers) who attended a teaching hospital centre and fulfilled the diagnostic criteria for DPB were ass essed prospectively far clinical, radiological, lung function, microbi ological, and other ''characteristic'' laboratory parameters. Results- Lung function assessment showed a typical obstructive pattern (n = 5) and air trapping (n = 7). Typical. bronchiolar infiltration by lymphoc ytes and plasma cells and accumulation of foamy macrophages in the int raluminal tissue were detected in open lung biopsy specimens (n = 2). Chest radiographs and high resolution computed tomographic scans revea led hyperinflation, diffuse nodules, bronchial thickening and dilatati on, peripheral hypoattenuation, and bronchiolectasis. Radiological imp rovement, manifest as a reduction in nodular density and branchial thi ckening, and persistence of other abnormalities such as air trapping w ere not accurately depicted by the classical Nakata or Akira classific ations. The other ''characteristic'' features such as HLA-B54, IgG sub class deficiency, raised CD4/CD8 T lymphocyte ratio, cold haemagglutin aemia, raised IgA, IgG, and rheumatoid factor were not present. Treatm ent with erythromycin led to excellent responses in symptoms, lung fun ction Indices, and the radiological picture. A review of the: non-Japa nese cases Japan in the literature reveals that this absence of typica l ''additional features'' in DPB might also be applicable to non-Japan ese patients. Conclusions-We report the only series of non-Japanese Mo ngoloid patients with well characterised DPB who had uncharacteristic investigation profiles. This experience should help other clinicians i n the investigation and management of DPB in non-Japanese patients.