Diffuse panbronchiolitis

Diffuse panbronchiolitis (DPB) is characterized by chronic airway infection with diffuse bilateral micronodular pulmonary lesions. DPB is mainly distributed in east Asian people. Studies on causes of the disease point to a genetic predisposition unique to Asians. The advent of low-dose, long-term macrolide therapy has changed disease prognosis. The mechanism of action is attributed to anti-inflammatory actions of 14-membered and 15-membered ring macrolides. Recently, the success of macrolide therapy in DPB has extended its application to the treatment of other chronic airway inflammatory diseases.     

Diffuse panbronchiolitis.

Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease, well recognised in Japan and principally affecting the respiratory bronchioles, causing a progressive suppurative and severe obstructive respiratory disorder. If left untreated, DPB progresses to bronchiectasis, respiratory failure and death. It was first described in the early 1960s. Subsequently, in 1969, the disease was named DPB to distinguish it from chronic bronchitis. "Diffuse" refers to the distribution of the lesions throughout both lungs, and "pan-" refers to the involvement of inflammation in all layers of the respiratory bronchioles. The distinctive imaging and histological features, the coexisting sinusitis, and the isolation of Haemophilus influenzae and Pseudomonas aeruginosa in the sputum enhance disease recognition. Histologically, DPB is characterised by chronic inflammation, localised mainly in the respiratory bronchioles and adjacent centrilobular regions, with characteristic interstitial accumulation of foamy histiocytes, neutrophils and lymphocyte infiltration. Neutrophils and T-lymphocytes, particularly CD8+ cells, together with the cytokines interleukin-8 and macrophage inflammatory protein-1, are believed to play key roles in the development of DPB. A significant improvement in the prognosis of this potentially fatal disease has been recently reported thanks to the use of long-term therapy with macrolide antibiotics, the effect of which is attributed to an anti-inflammatory and immunoregulatory action.     

Diffuse panbronchiolitis in China

OBJECTIVE: Diffuse panbronchiolitis (DPB) is a progressive inflammatory disease, well recognized in Japan, that is characterized by chronic sinusitis and obstructive small airway disease. A total of 40 patients with DPB in mainland China were reviewed in order to describe the epidemiology of DPB in China and to compare their clinical characteristics with those of cases reported in Japan. METHODOLOGY: A systematic search was conducted of the Chinese language literature published in mainland China from 1996 to 2003. The 40 subjects who fulfilled the diagnostic criteria for DPB were retrospectively analysed for clinical, physiological, radiological, and pathological features and their geographic distribution. RESULTS: The 40 patients included in this study (31 male and nine female) were distributed in 12 provinces and three other cities in China. All presented with chronic cough, sputum production, and 32 had exertional dyspnoea. All but three had a history of sinusitis. Lung function assessment showed a mixed obstructive-restrictive pattern in 30 cases. CXR revealed diffuse fine nodular shadows in all patients. Human leukocyte antigen-B54 was positive in five patients (n = 12). A total of 30 (75%) patients had been misdiagnosed prior to their diagnosis of DPB being made. CONCLUSIONS: DPB is not rare in China but its incidence is relatively low. Poor recognition and/or genetic factors may be the reason. The clinical, radiographic and histological features of Chinese patients resemble those described in Japanese patients.     

Diffuse panbronchiolitis preceding ulcerative colitis

To expand the clinical spectrum of ulcerative colitis-associated lung disease, we describe a patient with panbronchiolitis associated with ulcerative colitis. In addition to his having a distinctive pulmonary manifestation of this bowel disease, other noteworthy aspects of this patient's course include the onset of pulmonary symptoms several years prior to bowel manifestations and the partial resolution of his pulmonary symptoms following colectomy.     

老年弥漫性泛细支气管炎

弥漫性泛细支气管炎（diffuse panbronchiolitis,DPB）是主要累及呼吸性细支气管的慢性弥漫性炎性疾病,由日本的山中、本间、谷本等于1969年首次提出,逐步得到欧美医学界认可为独立的疾病。主要临床表现为反复咳嗽、咳痰、活动后呼吸困难。DPB起初在日本报道较多,逐步在韩国、中国也有报道,欧美地区报道病例目前仍不多见。本病发病年龄从10岁到80岁,40-50岁为发病高峰年龄段,     

Diffuse panbronchiolitis in Latin America

Diffuse panbronchiolitis (DPB) is a unusual form of bronchiolar disease that has been reported almost exclusively in Asians. We describe DPB in a non-Asian Brazilian citizen who has never traveled outside the country. The clinical, radiographic, and histologic features of this case resemble those described in Japanese patients. The present case shows that DPB, although rare in Western countries, is not a disease restricted to Asia. It always should be considered in the differential diagnosis of nodular radiographic opacities associated with airflow limitation, especially in non-smokers with a history of chronic sinusitis.     

弥漫性泛细支气管炎的诊治

目的探讨弥漫性泛细支气管炎（DPB）的临床特点及诊断方法。方法举例确诊为DPB患者的临床资料,并结合文献复习。结果DPB是一种以两肺弥漫性呼吸性细支气管及其周围的慢性炎症为特征的独立性疾病,临床表现为慢性咳嗽、咳痰、活动后呼吸困难,常合并铜绿假单胞菌感染和慢性鼻窦炎;胸部高分辨CT（HRCT）有助于诊断弥漫性泛细支气管炎,早期诊断及时给予大环内酯类药物规范化治疗,本病预后良好。结论对长期慢性咳嗽、咳痰,合并有慢性鼻窦炎病史的患者。应高度怀疑本病可能,应用小剂量红霉素或其他大环内酯类抗菌药物长期治疗,效果良好。     

弥漫性泛细支气管炎的免疫学发病机制

弥漫性泛细支气管炎(diffuse panbronchiolitis,DPB)患者的呼吸性细支气管区域有淋巴细胞、浆细胞、巨噬细胞浸润和聚集,支气管组织中的树突细胞异常以及支气管肺泡灌洗液CD4+/CD8+细胞比值增高.血冷凝集试验效价持续升高.IgA增高等提示DPB的发病可能与免疫功能紊乱有关.     

Diffuse panbronchiolitis: role of macrolides in therapy

Diffuse panbronchiolitis (DPB) is characterized by chronic sinobronchial infection and diffuse bilateral micronodular pulmonary lesions consisting of inflammatory cells. Studies on disease etiology point to a genetic predisposition unique to Asians. Early therapy for DPB was largely symptomatic. The advent of macrolide antibiotics, including erythromycin, roxithromycin and clarithromycin, has strikingly changed disease prognosis. Low-dose, long-term macrolide therapy for DPB originated from detailed observations of response to therapy in a single patient. The bactericidal activity of macrolides, particularly erythromycin, is not a significant factor for their clinical efficacy in DPB. Firstly, irrespective of bacterial clearance, clinical improvement is observed in patients treated with erythromycin. Secondly, even in cases with bacterial superinfection with Pseudomonas aeruginosa resistant to macrolides, treatment has proved effective. Thirdly, the recommended dosage of macrolides produces peak levels in tissue that are below the minimum inhibitory concentrations for major pathogenic bacteria that colonize the airway. In the last two decades, the possible mechanism underlying the effectiveness of macrolide therapy has been extensively studied. The proposed mechanism of action includes inhibition of excessive mucus and water secretion from the airway epithelium, inhibition of neutrophil accumulation in the large airway, inhibition of lymphocyte and macrophage accumulation around the small airway, and modulation of bacterial virulence. The great success of macrolide therapy in diffuse panbronchiolitis may extend its application to the treatment of other chronic inflammatory disorders. If the anti-inflammatory activity of macrolides is independent of their bactericidal effect, new anti-inflammatory macrolides without antimicrobial activity should be developed to minimize emergence of macrolide-resistant micro-organisms.     

Diffuse panbronchiolitis in two brothers with different clinical courses]

Diffuse panbronchiolitis in two brothers is reported. The elder brother aged 46, was admitted in May 1983 due to severe dyspnea and productive cough, which had gradually worsened over several years. He had severe hypoxemia and hypercapnia. He died at age 47 of respiratory failure due to pseudomonas infection despite antibiotic therapy. The younger brother, at age 41, was admitted in March 1983 due to fever, productive cough, and abnormal shadows on chest X-ray films. He showed mild hypoxemia and his symptoms improved with antibiotic treatment. Since then he has been followed as an outpatient for over 7 years while taking 400 mg of Erythromycin per day, and he has had no exacerbation. These two cases had different clinical courses despite the facts that both had similar conditions of chronic sinusitis and appeared to be exposed to no special environmental or occupational hazards. These facts suggest that not only intrinsic factors, such as defenselessness of airways, but extrinsic factors such as viral, mycoplasmal, or bacterial infection may act together on the mechanisms of the onset and progression of diffuse panbronchiolitis.     

Diffuse panbronchiolitis as a pulmonary complication in patients with adult T-cell leukemia

Forty-three cases of adult T-cell leukemia (ATL) admitted to our hospital between 1982 and 1987 were studied. Three of those were found to be complicated with diffuse panbronchiolitis (DPB). The incidence of DPB is considered to be significantly higher in patients with ATL. The three DPB-complicated cases composed one case each of the smoldering, chronic, and acute type of ATL. In each type, DPB preceded overt ATL and Candida albicans was found in sputa following detection for bacteria. The DPB complication apparently worsened the prognosis of the ATL patients. We have discussed a possible relationship between ATL and DPB.