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及其治疗进展

弥漫性泛细支气管炎（diffuse panbronchiolitis，DPB）是一种弥漫存在于两肺呼吸性细支气管区域的慢性炎症为特征，引起严重呼吸障碍的疾患。由于炎症病变弥漫性地分布并累及呼吸性细支气管壁的全层，故称之为弥漫性泛细支气管炎。主要症状为慢性咳嗽、咳痰、活动时气短。由于进行性慢性气道感染，常引起呼吸衰竭，多数预后不良，但随着红霉素疗法的应用，DPB的预后有了划时代的变化。笔者复习了有关文献，对本病及其治疗进展综述如下：     

大环内酯类抗生素对T细胞巨噬细胞炎性蛋白-1α表达的影响

弥漫性泛细支气管炎(DPB)是以弥漫存在于两肺呼吸性细支气管慢性炎症为特征并可引起严重呼吸障碍的一种疾病.大环内酯类抗生素治疗DPB可获得良好疗效,但治疗机制尚不完全清楚.     

弥漫性泛细支气管炎6例临床分析

目的:探讨弥漫性泛细支气管炎(DPB)的临床特征、诊断和治疗。方法:回顾性分析我科收治的6例DPB患者的临床资料。结果:6例DPB患者中男性1例,女性5例;均有慢性鼻窦炎;临床表现为咳嗽、咳痰及活动性气促,体格检查肺部均可闻及湿罗音,1例可闻及哮鸣音;肺功能第1秒末用力呼气量/用力肺活量70%且均有低氧血症;1例血清冷凝集试验效价1:64;所有患者胸部高分辨CT显示,沿支气管血管束分布的双肺弥漫性小叶中心结节影,伴树芽征,2例可见支气管扩张;1例行气管镜检查,肺泡灌洗及肺组织活检中淋巴细胞增多;经应用大环内酯类抗生素治疗,6例DPB患者临床症状均改善。结论:了解DPB的临床特征有助于早期诊断、早期治疗,改善预后。     

同意诊断弥漫性泛细支气管炎不应过分强调病理活检

有学者提出,在临床诊断弥漫性泛细支气管炎(DPB)时,满足临床诊断的6条标准即可,不一定依赖于病理诊断,因为肺活检的风险大于其可能的获益[1].笔者同意上述观点,理由如下:(1)不依赖于病理诊断并不会显著增加DPB的报告例数.     

类风湿性关节炎伴弥漫性泛细支气管炎1例报告并文献复习

弥漫性泛细支气管炎(DPB)是弥漫性存在于两肺呼吸性细支气管区域的一种慢性炎症为特征的独立性疾病。近年来,多有文献报道。常被误诊为支气管扩张,慢性支气管炎等,而贻误治疗。本文结合我院2012年以来收治的1例类风湿性关节炎伴弥漫性泛细支气管炎病例进行总结,提高对本病的认识。     

北方汉族弥漫性泛细支气管炎患者人类白细胞抗原Ⅰ类抗原的特点

弥漫性泛细支气管炎（diffuse panbronchiolitis, DPB）是一种以弥漫存在于两肺呼吸性细支气管区域的、以慢性炎症为特征的疾病，发病具有明显的种族差异，主要聚集于东亚国家，且84.8％的患者合并慢性鼻窦炎或鼻息肉，其中最引人注目的是有关人类白细胞抗原（human leukocyte antigen，HLA）的研究。日本口剖和韩国学者分别对本国的DPB患者进行HIA检测，     

弥漫性泛细支气管炎4例

弥漫性泛细支气管炎（diffusepanbronchiolitis,DPB）是一种以弥漫存在于两肺呼吸性细支气管区域的慢性炎症为特征,引起严重呼吸障碍而病因尚属不明的一种疾病。突出的临床表现是咳嗽、咳痰及活动时气促。临床上常易误诊为慢性阻塞性肺病、支气管扩张等疾病。我院2009年5月至2013年6月收治4例DPB患者资料,报道如下。     

弥漫性泛细支气管炎的CT诊断及鉴别诊断

目的探讨CT对弥漫性泛细支气管炎(DPB)的诊断及鉴别诊断价值。方法总结和分析42例DPB患者胸部及鼻窦的CT表现,其中病理活检确诊5例,临床诊断确诊37例。结果 DPB胸部CT主要表现为:①弥漫性细粟粒样影伴树芽征;②小气道细支气管扩张;③炎性斑片影。鼻窦CT检查多伴随有副鼻窦炎表现(37例,88.1%),最常累及上颌窦。结论DPB胸部CT表现具有一定的特征性,多数患者合并副鼻窦炎,有助于DPB的早期诊断和鉴别诊断。     

冷凝集试验在弥漫性泛细支气管炎诊断中的价值

弥漫性泛细支气管炎(diffuse panbronchiolitis,DPB)是以慢性鼻窦炎、两肺弥漫呼吸性细支气管炎为特点的独立疾病[1].     

Childhood diffuse panbronchiolitis: a case report

Diffuse panbronchiolitis (DPB) is a chronic, potentially life-threatening lower respiratory tract disease that is particularly common in Japanese people. If left untreated, it progresses to bronchiectasis, respiratory failure, and death. Lack of familiarity with DPB in the non-Far East may result in a failure to correctly diagnose and treat this disorder. We describe a child with DPB. We suggest that DPB is a sinopulmonary disease that is not exclusive to the Asian population and to adults. Its clinical and radiological features should be better known by pediatric pulmonary physicians.     

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.     

Therapy against intractable respiratory infections with Pseudomonas aeruginosa

Patients with diffuse panbronchiolitis (DPB) are frequently affected by Pseudomonas aeruginosa superinfection. To elucidate the predisposing factors of Pseudomonas aeruginosa superinfection in patients with DPB, we analyzed the age of onset, duration of the disease, chest X-ray findings, blood gas levels, lung function, and bacteriological findings. These data were compared with those of patients who had not developed Pseudomonas aeruginosa superinfection. The administration of antibiotics and corticosteroids did not influence the incidence of Pseudomonas aeruginosa superinfection in DPB patients. The patients with long duration, more severe lung function and more deteriorated roentgenological findings developed Pseudomonas aeruginosa superinfection more easily. These infections in the lower respiratory tract significantly affect the prognosis of DPB patients. Using long-term administrations of a new quinolone antibacterial agent against DPB, acute exacerbations were controlled in some patients and the frequency of their admission to hospital was lessened. A multicomponent vaccine raised antibody titers against OEP, elastase, protease and exotoxin in DPB patients. Further clinical investigations are under way in our hospital to confirm the clinical usefulness of the Pseudomonas aeruginosa multicomponent vaccine in DPB from prophylactic and therapeutic points of view.     

Deterioration of Asthma in a Patient With Diffuse Panbronchiolitis (DPB) After Macrolide Therapy

Diffuse panbronchiolitis (DPB), an important cause of progressive obstructive lung disease in the Far East, is a distinctive sinobronchial syndrome with characteristic radiologic and histologic features. Asthma is a chronic inflammatory disease characterized by airway narrowing. The major inflammatory cells involved in the pathogenesis of asthma are type 2 helper T (Th2) cells, eosinophils, and mast cells. The authors’ patient was diagnosed with DPB and asthma. Although macrolide therapy led to the disappearance of the radiologic abnormalities indicating centrilobular nodular lesions, the respiratory symptoms and pulmonary function worsened. Administration of inhaled corticosteroids improved the respiratory symptoms and pulmonary function. To the authors’ knowledge, no case of DPB with asthma has been reported in the English-language literature.     

The effects of azithromycin on patients with diffuse panbronchiolitis: a retrospective study of 29 cases

Background

Diffuse panbronchiolitis (DPB), a chronic inflammatory disease of the airway, is treated with macrolide antibiotics. The ability of azithromycin to improve DPB prognosis, as detected by high-resolution computed tomography (HRCT) scans and lung function tests, has not been studied in a large retrospective of patients. Our study aims to investigate the effects of azithromycin on patients with DPB using lung function tests and radiologic images.

Methods

Twenty-nine patients with DPB were studied; their medical records were collected and analyzed retrospectively. Patients studied were hospitalized in the respiratory department of the Yixing Hospital, affiliated with Jiangsu University. Azithromycin was administered for 6-17 months. Changes in lung function and HRCT scans after treatment with azithromycin for six months were compared with pre-treatment values and images respectively.

Results

Azithromycin therapy for six months resulted in rapid improvements in lung function, demonstrated by forced expiratory volume in one second (FEV1.0%), forced expiratory volume in one second over the forced vital capacity (FEV1.0/FVC), and forced expiratory volume with 75% vital capacity (FEF75%) values. In addition, improvements were seen in small nodular shadows, dilated peripheral bronchi, bronchial wall thickening, and tree-in-bud pattern, as detected by chest HRCT scans.

Conclusions

Long-term therapy with azithromycin is effective for patients with DPB.KEYWORDS : Diffuse panbronchiolitis (DPB), azithromycin, lung function, high-resolution computed tomography (HRCT)     

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.     

A case of diffuse panbronchiolitis (DPB) in a patient positive for HTLV-1

Diffuse panbronchiolitis (DPB), an important cause of progressive obstructive lung disease in the Far East, represents a distinctive sinobronchial syndrome with typical radiologic and histologic features. Human T-cell lymphotrophic virus (HTLV-1) is a retrovirus that clinically and experimentally suppresses T-cell function and immune responses. The clinical and immunologic features of DPB in HTLV-1 carriers are unclear, because DPB and HTLV-1 endemic areas around the world are mostly non-overlapping. However, both diseases are endemic in Japan. We present a patient with DPB positive for HTLV-1 whose cellular and humoral immune responses were markedly impaired. Six y after diagnosis of DPB, the patient developed respiratory failure and died in spite of treatment with clarithromycin.     

Diffuse panbronchiolitis: a treatable sinobronchial disease in need of recognition in the United States

Diffuse panbronchiolitis (DPB) is a progressive inflammatory disease, well recognized in Japan, characterized by sinusitis and obstructive small airway disease; if left untreated, it progresses to bronchiectasis, respiratory failure, and death. Treatment using low-dose erythromycin has proven to be highly efficacious. Lack of familiarity with DPB in the United States may result in the failure to correctly diagnose and treat this disorder. We describe a Cambodian man in whom the characteristic imaging and histologic features of DPB were elicited but not recognized in spite of evaluation at a referral center. When DPB was diagnosed 6 years later, he was in respiratory failure, but made an excellent recovery once erythromycin therapy was instituted. We report this case to increase physician awareness of DPB as a cause of sinobronchial disease and discuss its diagnostic features so that the disease is recognized and treated without delay.     

Clinical evaluation of serum sialyl Lewis X-i (SLX) in diffuse panbronchiolitis

Clinical comparison of SLX with other tumor-associated antigens such as CA19-9, CA125, and CEA, was made in benign respiratory diseases including diffuse panbronchiolitis (DPB), bronchiectasis (BE), bronchial asthma (BA), and pulmonary emphysema (PE). Sensitivities of each marker (greater than 38.0 U/ml) on DPB were 79.4% in SLX, 68.0% in CA19-1, 46.7% in CA125, and 35.7% in CEA. Serum levels of SLX in DPB and BE were significantly higher than those in BA and PE. There seemed to be no relationship between serum levels of SLX and CRP, ESR, and the volume of sputum. Immunohistochemical studies showed positive staining on the surface of respiratory bronchioles and alveolar walls in DPB and these findings were not observed in normal lung tissues. We consider that the high value of serum levels of SLX in DPB can be explained by these findings.     

A mechanism of usefulness of treatment with erythromycin for diffuse panbronchiolitis]

The clinical usefulness of treatment with erythromycin for diffuse panbronchiolitis (DPB) patients have been well reported to date, although its mechanisms remain unknown. In a previous study, we demonstrated that DPB patients were found to have large percentages of neutrophils in their lower respiratory tracts and suggested that neutrophils play an important role in the pathogenesis of DPB (Chest 99: 917-923, 1990). While in vitro and in vivo studies have demonstrated that erythromycin decreases neutrophils directed migration. In the present study, we performed bronchoalveolar lavage (BAL) on eight patients with DPB to clarify the cell populations in the lower respiratory tract before and after treatment with erythromycin. Neutrophils percentages in BAL fluid were significantly reduced after treatment with erythromycin (54.3 +/- 22.4% to 13.1 +/- 13.5%, p less than 0.01). No significant variance of BAL lymphocyte percentages was demonstrated after treatment with erythromycin. These results suggest that a main mechanism of clinical usefulness of treatment with erythromycin for DPB may be inhibition of neutrophils migration into the lower respiratory tract instead of intrapulmonary bactericidal activity against organisms.