胸膜肺弹力纤维增生症研究进展

Progress in research of pleuroparenchymal fibroelastosis

胸膜肺弹力纤维增生症(PPFE)是近年来被认识的一种间质性肺疾病类型,临床罕见,容易误诊。活动后气促、咳嗽、胸痛、消瘦、体质量进行性下降、扁平胸廓、胸膜增厚、胸膜下纤维化、限制性通气功能障碍以及气胸发生率高为患者常见的临床特征。PPFE的治疗尚未达成共识,预后差,药物治疗效果欠佳,肺移植是PPFE患者疾病进展至终末期的治疗选择。本文对PPFE的病因、临床表现、诊断及治疗进行综述,以提高临床医师对该病的诊疗水平。

Pleuroparenchymal fibroelastosis(PPFE)known in recent years is a type of interstitial lung disease,which was easily misdiagnosed due to rare in clinical.The common clinical features of this population include shortness of breath after exercise,cough,chest pain,emaciation,progressive decline of body mass,flat chest,pleural thickening,subpleural fibrosis,restrictive ventilation dysfuction,and high incidence of pneumothorax.There is no consensus on the treatment of PPFE,it has a poor prognosis and poor treatment effect by drug,lung transplantation is the therapeutic option for terminal PPFE patients.This article reviews the progress in the understanding of the etiology,clinical manifestations,diagnosis,and treatment of PPFE to improve the diagnosis and treatment of this disease for clinician.