弥漫性泛细支气管炎临床特征研究

Study of clinical features of diffuse panbrochiolitis

目的研究弥漫性泛细支气管炎的临床特点,提高对该疾病的认知和诊断治疗水平。方法收集2011年1月~2016年10月我院收治的14例弥漫性泛细支气管炎患者的临床资料,结合相关文献,分析该疾病的临床表现、肺功能、影像特点、相关实验室检查和治疗方法。结果 14例患者均有咳嗽咳痰,活动时气短10例,听诊肺部均闻及湿啰音。12例患者有明确的慢性鼻窦炎病史。胸部CT均提示弥漫性分布的小叶中心性粟粒状小结节影伴树芽征。肺功能提示阻塞性通气功能障碍,小气道功能障碍尤为明显,1秒用力呼气容积占预计值百分比(FEV1%)为(46.7±7.5)%。动脉血氧分压均低于80 mm Hg,平均值为(69.1±7.6)mm Hg。10例DPB患者行血冷凝集试验,均阴性。初次就诊误诊率高达100.0%,常被误诊为肺炎、支气管扩张、哮喘、慢性阻塞性肺病。予以大环内酯类抗生素治疗症状均改善。结论 DPB患者临床表现特异性不高,易误诊。提高临床医师的认知度,早发现早诊断早治疗改善预后。血冷凝集试验在中国DPB患者阳性率低,提示该指标可能并不适用中国DPB的诊断。

Objective To study the clinical features of diffuse panbronchiolitis（DPB）, in order to improve the understanding of the disease, diagnosis and treatment. Methods The clinical data and imaging features, pulmonary function,laboratory examination and treatment of 14 patients of DPB from January 2011 to October 2016 in our hospital were retrospectively analyzed, and literatures were reviewed. Results Repeatedly cough, sputum and crackle on auscultation were found in all of 14 patients, shortness of breath was found in 10 patients. Chronic sinusitis was found in 12 patients. Chest computed tomography showed diffuse centrilobular nodules and tree-in-bud patterns in all patients. Pulmonary function prompted obstructive ventilation dysfunction, small airway dysfunction particularly. The average FEV1% was（46.7±7.5）%. Arterial blood oxygen partial pressure were all below 80 mm Hg, the average Pa O2 was（69.1±7.6） mm Hg. Blood cold hemoagglutinin（CHA） of 10 patients with DPB were all negative. The initial treatment misdiagnosis rate was as high as 100.0%. Most of them had been misdiagnosed as pneumonia, bronchiectasis asthma, chronic obstructive pulmonary. After regular macrolides antibiotics treatment, their clinical symptoms were improved significantly. Conclusion Clinical specificity of DPB is not high, so it is easy to make a mistake diagnosis. Raising clinician awareness, early detection and early diagnosis and early treatment improve prognosis. The positive rate of CHA in Chinese DPB is low, the result suggests that CHA may not be applies to the diagnosis of Chinese DPB.