Clinical profiles of Chinese patients with diffuse panbronchiolitis

Abstract

Background-DiVuse panbronchiolitis (DPB), characterised by progressive sinobronchial sepsis, is well characterised in Japanese subjects but not in other ethnic groups. The experience with DPB in seven Chinese patients is described and the clinical profiles compared with those of Japanese subjects. Methods-Seven Chinese patients (three women; mean (SD) age 48(18.6) years, all never smokers) who attended a teaching hospital centre and fulfilled the diagnostic criteria for DPB were assessed prospectively for clinical, radiological, lung function, microbiological, and other "characteristic" laboratory parameters. Results-Lungfunction assessment showed a typical obstructive pattern (n = 5) and air trapping (n = 7). Typical bronchiolar infiltration by lymphocytes and plasma cells and accumulation of foamy macrophages in the intraluminal tissue were detected in open lung biopsy specimens (n = 2). Chest radiographs and high resolution computed tomographic scans revealed hyperinflation, diVuse nodules, bronchial thickening and dilatation, peripheral hypoattenuation, and bronchiolectasis. Radiological improvement, manifest as a reduction in nodular density and bronchial thickening, and persistence of other abnormalities such as air trapping were not accurately depicted by the classical Nakata or Akira classifications. The other "characteristic" features such as HLA-B54, IgG subclass deficiency, raised CD4/CD8 T lymphocyte ratio, cold haemagglutinaemia, raised IgA, IgG, and rheumatoid factor were not present. Treatment with erythromycin led to excellent responses in symptoms, lung function indices, and the radiological picture. A review of the non-Japanese cases in the literature reveals that this absence of typical "additional features" in DPB might also be applicable to non-Japanese patients. Conclusions-We report the only series of non-Japanese Mongoloid patients with well characterised DPB who had uncharacteristic investigation profiles. This experience should help other clinicians in the investigation and management of DPB in non-Japanese patients. (Thorax 1998;53:274-280)

Molecular cloning of two novel mucin-like genes in the disease-susceptibility locus for diffuse panbronchiolitis

“…DPB was Wrst described in Japanese patients (Homma et al 1983) and subsequently in Koreans (Kim et al 1992) and Chinese (Chu et al 1992;Tsang et al 1998). Sugiyama et al (1990) reported that the frequency of human leukocyte antigen (HLA)-B54 was much higher in Japanese DPB patients than that in the normal population.…”

Effect of Azithromycin on Patients with Diffuse Panbronchiolitis: Retrospective Study of 51 Cases

“…It was originally reported in Japan (2). However, an increasing number of cases have been reported in Europe (3,4) and America (5)(6)(7)(8)(9), as well as in Asia (10), including Thailand (11), India (12), and China (13)(14)(15)(16)(17)(18)(19)(20).…”

Bronquiectasias tras colectomía en paciente con colitis ulcerosa y mutación del factor V Leiden

“…Suelen tener un curso paralelo al de la EII, con brotes de síntomas respiratorios junto con brotes de la enfermedad digestiva, hecho que ha llevado a plantear la hipótesis de que la enfermedad podría producirse en el pulmón al tener tanto el tubo digestivo como el pulmón un mismo origen embrionario; este hecho se ve reforzado en el caso de las bronquiectasias tras colectomía, donde, tras quitar el órgano diana del proceso inflamatorio, el aparato respiratorio pasa a ser el siguiente en presentar el daño inflamatorio 9 . Las bronquiectasias suelen ser estériles y sólo demuestran un aumento de los neutrófilos en el lavado broncoalveolar 10,11 .…”