32例弥漫性泛细支气管炎的临床特征

Clinical characteristics of 32 cases of diffuse panbronchiolitis

目的:弥漫性泛细支气管炎(diffuse panbronchiolitis,DPB)是一种慢性气道炎症,临床表现缺乏特异性,漏诊及误诊率高。本研究旨在通过总结DPB的临床特征及治疗情况,提高临床医师对该病的认识及诊断水平。方法:收集2013年3月1日至2022年3月1日中南大学湘雅二医院收治的32例DPB患者的临床资料,分析患者的基础信息、临床表现、实验室检查、肺功能、影像学检查、治疗及转归情况。结果:32例DPB患者的男女比例为1.67꞉1,中位发病年龄为26.5(11.0~69.0)岁,中位确诊年龄为47.5(16.0~77.0)岁。32例患者入院时均有咳嗽、咳痰,26例存在活动后气促。14例患者血冷凝集试验效价≥1꞉64。31例患者行肺功能检查,18例表现为混合型肺通气功能障碍,12例表现为阻塞型肺通气功能,1例肺通气功能正常。31例肺部CT表现为弥漫性小结节影。32例患者均接受大环内酯类药物治疗,出院时31例患者临床症状好转,20例患者复查血气分析提示氧分压、血氧饱和度较前好转。12例患者治疗后复查肺部CT,6例提示弥漫性小结节影较前减少,5例与治疗前相比无明显变化,1例提示弥漫性小结节影较前增多,提示疾病进展。7例患者治疗后行肺功能检查,治疗前后第1秒用力呼气量(forced expiratory volume in one second,FEV_(1))及FEV_(1)/用力肺活量有改善,用力呼出25%肺活量时最大用力呼气流量无明显变化。结论:DPB临床表现无特异性,早诊断、早治疗对患者的预后至关重要。

Objective:Diffuse panbronchiolitis(DPB)is a chronic airway inflammation with low specificity and its diagnosis is often missed or delayed.This study aims to summarize the clinical characteristics and treatment of DPB in order to improve the understanding and diagnosis of the disease.Methods:The clinical data of 32 DPB patients were collected,analyzed and summarized from March 1,2013 to March 1,2022 in the Second Xiangya Hospital of Central South University.The basic information,clinical manifestations,laboratory tests,pulmonary function,imaging tests,treatment,and regression of patients were analyzed.Results:A total of 32 patients were enrolled in the final analysis,with a male-to-female ratio at 1.67.The median age at symptom onset was 26.5(11.0−69.0)years,and the median age of diagnosis was 47.5(16.0−77.0)years.All patients presented with chronic cough and copious sputum production.A total of 26 patients had post activity shortness of breath and 14 patients had a positive result(blood cold agglutination test titer≥1꞉64).Pulmonary function examination was performed in 31 patients,18 patients showed mixed pulmonary ventilation dysfunction,12 patients showed obstructive pulmonary ventilation,and 1 patient had normal pulmonary ventilation function.A total of 31 patients had a bilateral,diffuse,small nodule pattern on chest CT.All patients were treated with macrolides.A total of 31 patients showed improvement,and 20 patients showed improvement in partial pressure of oxygen and blood oxygen saturation compared with before at discharge.A total of 12 patients were re-examined by chest CT after completing macrolides treatment,6 cases showed less diffuse nodules,5 cases showed no significant changes,and 1 case showed more diffuse nodules,which indicated the disease progression.Seven patients received pulmonary function tests after completing macrolides treatment,forced expiratory volume in one second(FEV_(1))and FEV_(1)/forced vital capacitywere improved,but forced expiratory flow at 25%of vital capacity did not change significantly.Conclusion:The clinical manifestations of DPB are nonspecific.Early diagnosis and treatment are very important for the prognosis of patients.