弥漫性泛细支气管炎的诊断:18例分析

目的 探讨弥漫性泛细支气管炎(diffuse panbronchiolitis,DPB)的临床特点、影像学表现、诊断和鉴别诊断.方法 对在北京协和医院诊断为DPB的18例患者进行回顾分析.结果 病理确诊10例,临床诊断14例.18例均以慢性咳嗽、咳痰及活动气促为主诉.14例有肺部哕音.15例有鼻窦炎.肺功能以阻塞性通气功能障碍为主.本组病例冷凝集试验阳性率低.所有胸部影像见弥漫小结节影,大部分伴支气管扩张;影像学分型和临床严重程度无明确相关性.初诊误诊率100%.结论 本组DPB患者有典型临床表现,但某些临床特点有别于日本患者.DPB影像学有特征性表现.DPB误诊率高,应提高对其认识.

Diagnosis of diffuse panbronchiolitis:clinical analysis of 18 cases

ObjectiveTo investigate the clinical features, radiological findings for diffuse panbronchiolitis(DPB) in Chinese patients. Methods Eighteen patients who attended Peking Union Medical College Hospital and fulfilled the Japanese revised clinical diagnostic criteria for DPB were assessed prospectively for clinical,laboratory parameters and radiological examination. Results Of the 18 cases, 10 were pathologically proven cases, 8 were clinical proven. All the patients had clinical manifestations of persistent cough,sputum production and exertion dyspnea,only 1 had hemoptysis. 14 had coarse rale, 15 had chronic sinusitis. Most cases had obstructive pulmonary dysfunction, with hypoxaemia. The other "characteristic" features such as cold haemagglutinaemia, raised CD4/CD8 T lymphocyte ratio, raised IgA and IgG were not present. All cases had centribular micronodules on chest CT, most with bronchiectasis. There were no correlation between radiological classification and clinical stages in these patients. Furthermore,almost all cases were missed or mistaken for other diseases. Conclusions All DPB cases had typical clinical features, but were deficient in some "additional features". The radiological manifestation plays an important role in making diagnosis of DPB,but its value in clinical severity is not sure. DPB is easy to be misdiagnosed,so should be paid more attention.