特发性胸膜肺弹力纤维增生症临床特点及文献复习  

Idiopathic pleuroparenehymal fibroelastosis:five case reports and review of literature

作　　者：桂贤华[1] 邱玉英[1] 陈婷婷 李慧[1] 代静泓[1] 蔡后荣[1] 肖永龙[1] 曹敏[1] Gui Xianhua;Qiu Yuying;Chen Tingting;Li Hui;Dai Jinghong;Cai Hourong;Xiao Yonglong;Cao Min(Department of Respiratory and Critical Care Medicine,Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University,Nanjing 210008,China;Department of Pathology,Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University,Nanjing 210008,China)

机构地区：[1]南京大学医学院附属鼓楼医院呼吸内科,南京210008 [2]南京大学医学院附属鼓楼医院病理科,南京210008

出　　处：《中华结核和呼吸杂志》2022年第5期453-459,共7页Chinese Journal of Tuberculosis and Respiratory Diseases

摘　　要：目的提高对特发性胸膜肺弹力纤维增生症(IPPFE)的临床表现、胸部影像及病理组织学的认识。方法回顾性分析我院2018至2021年收治的5例IPPFE患者临床资料,其中男3例,女2例,年龄30~70岁,均经病理证实,分析其临床、影像学和病理特点。以“胸膜肺弹力纤维增生症”为中文关键词,“pleuroparenchymal fibroelastosis”为英文关键词,在万方、维普、中国知网以及PubMed数据库检索文献,检索时间截至2021年5月30日。阅读相关文献,总结IPPFE的临床特点。结果5例IPPFE患者从出现咳嗽气喘至诊断时间为9个月至3年,主要临床表现为咳嗽、活动后呼吸困难。胸部CT主要表现为双侧胸膜增厚,双上肺及胸膜下为主的实变影,伴牵拉性支气管扩张,CT引导下经皮肺活检或外科肺活检病理表现为特征性的胸膜及其下区域内密集分布的弹力纤维和胶原纤维,弹力纤维染色阳性,符合IPPFE。1例患者经小剂量激素治疗,2例患者未给予特殊治疗,2例患者口服吡非尼酮治疗,3例患者病情稳定。文献检索获得中文文献4篇,英文文献229篇,除外综述,资料不详及无病理诊断结果的文献后,最终对305例患者的进行总结。结论IPPFE的病理表现独特,但临床报道较少,可能存在漏诊或误诊,需要提高对IPPFE特征的认识,对指导临床和治疗、判断预后非常有益。Objective To raise the awareness of idiopathic pleuroparenehymal fibroelastosis(iPPFE)through investigating the clinical,radiographic and pathological features.Methods Five cases of iPPFE proved by pathology.The clinical data were studied respectively,and the relevant literature was reviewed.Results All the cases of iPPFE were manifested by cough and dyspnea.The patients including 3 males and 2 females,aged from 30 to 70 years Chest CT scan showed pleural thickening,subpleural consolidation in both upper lungs complicated with tractive bronchiectasis.Computed tomography-guided percutaneous lung biopsy or surgical lung were performed and the same pathological showed pleura and subpleural dense elastic and collagen fibers.The elastic fibers stain was also positive,which was consistent with PPFE.One patient received low-dose corticosteroid,two received pirfenidone therapy,the others received no treatment.Three patients were stable during the follow-up.Conclusions iPPFE has characteristic pathological features.However,the number of clinically reported cases is low due to missed diagnosis or misdiagnosed.Improving the understanding of features of iPPFE is helpful for the dianosis,therapy,and prognosis of this disease.

关 键 词：胸膜肺弹力纤维增生症 特发性间质性肺炎 外科肺活检 

分 类 号：R561[医药卫生—呼吸系统]