弥漫性泛细支气管炎的诊治现状

Status of Diagnosis and Treatment of Diffuse Panbronchiolitis

弥漫性泛细支气管炎(DPB)是一种少见的具有特定遗传背景的疾病,主要影响东亚人群,其特征为呼吸道细支气管慢性炎症。主要临床表现为持续性、进展性咳嗽、咳痰,活动后呼吸困难,伴有反复顽固性肺部感染及支气管扩张;患者多合并慢性鼻窦炎。典型CT表现为弥漫性小叶中心结节影伴"树芽征"。大环内酯类药物具有独特的抗炎及免疫调节作用,成为治疗DBP的基础药物。尽早诊断,长期应用小剂量大环内酯类药物治疗,可显著改善预后,甚至治愈。

Diffuse panbronchiolitis（DPB）,a rare genetic disease predominantly affecting East Asians,is characterized by chronic respiratory bronchiolic inflammation. The common symptoms are persistent cough,sputum,exertional dyspnea,refractory lung infections,and bronchiectasis,with the coexisting sinusitis. Typical CT features are diffuse lobular center nodules with ＂ tree bud signs＂. Low-dose,long-term treatment with macrolides,the effect of which is attributed to an anti-inflammatory and immunoregulatory action,has been the basic drugs for DPB patients. Early diagnosis and long-term application of low-dose macrolides can significantly improve the prognosis and even cure the patients.