中文摘要：

目的分析应用以阿奇霉素为主的综合措施治疗51例弥漫性泛细支气管炎（DPB）的疗效。方法选择同济大学附属上海市肺科医院2001年7月1日—2007年4月31日期间确诊为DPB的患者51例,并随访至2008年1月28日。采取以阿奇霉素为主的大环内酯类抗生素联合其他措施的综合治疗,包括阿奇霉素、糖皮质激素、针对感染致病菌的抗生素、氧疗及对症、支持疗法等。观察本组病例治疗前后的病情变化,包括临床症状、影像学表现、肺功能指标、动脉血气分析、痰菌检查等。结果51例患者中,男30例,女21例,男女比例为1.43∶1。年龄10～80岁,平均年龄为（51.8±15.6）岁。从起病到确诊的时间为10 d～40年,中位确诊时间为10年。所有病例均符合日本厚生省制定的DPB临床确诊标准,其中5例经肺活组织检查证实。96.1%（49/51）有副鼻窦炎,51.0%（26/51）继发支气管扩张,9.8%（5/51）伴肺源性心脏病,7.8%（4/51）合并支气管哮喘（7.8%）,9.8%（5/51）伴发高血压,2.0%（1/51）伴类风湿关节炎,2.0%（1/51）伴胸腺增生。人类白细胞抗原（HLA）-B54阳性率为25.0%（3/12）,冷凝集试验阳性率为17.6%（9/51）。本组DPB患者合并细菌感染,以铜绿假单胞菌阳性率最高（17/51,33.3%）,但阴转率仅为17.6%;其次为副流感嗜血杆菌（5/51,9.8%）,第3位是肺炎克雷伯菌（3/51,5.9%）。14例患者痊愈;34例好转;1例患者因依从性不佳,不规则短期用药而未愈;2例患者初期治疗后好转,1例出院后1年死于糖尿病酮症酸中毒,另1例出院后3个月死于心肌梗死。5年生存率达95.6%。结论DPB在中国并不少见,以阿奇霉素为主的综合治疗效果显著。

英文摘要：

Objective To analyze the outcomes of azithromycin-based comprehensive treatment for 51 patients with diffuse panbronchiolitis （DPB）. Methods Totally 51 DPB patients （30 males and 21 females, mean age 54 years）, who were treated with azithromycin in our hospital from Jul. 1, 2001 to Apr. 30, 2007, were included in the present study and were followed up to Jan. 28, 2008. The patients received comprehensive treatment using macrolides like Azithromycin, together with azithromycin, glucorcoticord, antibiotics, oxygen therapy, and supportive therapy, etc. The clinical, radiological characteristics, arterial gas analysis, lung function, microbiological findings, response to the therapy, and prognosis were observed before and after treatment. Results All patients presented with chronic cough and 50 （98.0%） with copious purulent sputum production; 49（96.1% ） had dyspnea on exertion; 33（64.7% ） had wheezes; and 49（96.1% ） had end inspiratory crackles in their chest as initial presentation. Twenty-five patients （49.0%） had emphysema; 49 （96.1%） had chronic sinusitis ~ 14 （27.5 % ） had smoking history ; 26 （51.0 % ） had bronchoectasis ; 5 （9.8 % ） had cor pulmonale 4（7.8% ）had asthma at same time; and 2 （a sister and an younger brother）came from the same family. Only 9 patients （17.6%） had elevated cold haemagglutinin titers and 5（9.8% ） had lung biopsy specimens. Three of 12 subjects （25.0 % ） had positive expression of human leukocyte antigen-B54 （HLA-B54）. Seventeen （33.3 % 〉 had positive sputum Pseudomonas aeruginosa. Thirty （58.8%） were misdiagnosed as having bronchoectasis and 23 （45.1% ） were misdiagnosed as having chronic bronchitis initially. Fourteen （27.5%）patients recovered entirely after therapy, but 2 of them relapsed after stopping treatment, and improved when given therapy again. Thirty-six cases were improved, but 2 of them died from other diseases during following up. The 5-year-survival rate was 95.6%. Conclusion DPB patien