摘要目的 总结分析胸膜肺弹力纤维增生症(pleuroparenehymal fibroelastosis,PPFE)患者的临床、影像和病理学特点,提高临床诊治水平.方法 回顾性分析北京医院收治的1例病理诊断的PPFE患者的临床资料,并进行相关文献复习.以"胸膜肺弹力纤维增生症"为检索词,检索万方数据库和同方期刊数据库;以"pleuroparenehymal fibroelastosis"为检索词,检索PubMed数据库,检索2017年10月之前报道的所有中文和英文文献.结果 患者男,46岁,咳嗽、活动后气短.胸部CT示双上胸廓变小,双肺尖胸膜增厚并胸膜下实变影,伴牵引性支气管扩张.曾反复抗结核治疗,疗程共3年,病情进展.行CT引导下肺穿刺,病理结果示胸膜肺弹力纤维增生症.文献复习共检索到国内外病例132例(其中中文文献报道1例),其中88例由病理确诊且资料详实.结合本例共纳入89例,男48例,女41例,年龄13~85岁,平均(50±17)岁.常见的症状为呼吸困难55例(62%),咳嗽52例(58%),反复呼吸道感染15例(17%).胸部影像学表现为胸膜增厚77例(87%),反复气胸46例(52%),牵张性支气管扩张27例(30%),胸膜下实变影18例(20%).89例均经组织活检病理确诊.34例接受糖皮质激素治疗,5例行肺移植.89例患者随访时间为4~84个月,随访期间40例因疾病进展死亡.结论 胸膜肺弹力纤维增生症是一种少见病,病变以双上肺为主,确诊要依据病理.临床需与肺结核或陈旧性肺结核、石棉肺、结缔组织疾病相关性肺病及药物性肺损伤鉴别.治疗方案尚未达成共识.该病预后不佳.

abstractsTo analyze the clinical, imaging and pathological features of Pleuroparenehymal fibroelastosis (PPFE). Methods The clinieal data of a patient diagnosed as PPFE admitted in department of Respiratory and Critical Care Medicine, Beijing Hospital in April 2017 were reported and the related literatures were reviewed. With"pleuroparenehymal fibroelastosis"as the search terms,and the search time before October 1st 2017 for Wanfangdata,China National Knowledge Infrastructure (CNKI),and PubMed.Results The patient was a 46?year?old male presented with cough, shortness of breath after exercise. A CT scan of the chest revealed bilateral, irregular pleural thickening with upper lobe predominance. After 3 years of antituberculosis treatment, the disease progressed. A diagnosis of pleuroparenehymal fibroelastosis (PPFE) was confirmed by CT guided lung biopsy. A total of 132 cases were reported (including 1 case in Chinese). 88 of them were confirmed by pathology with detailed data.Clinical data of 89 reported cases with PPFE including 48 males and 41 females aged 13 to 85 years were enrolled and analyzed in the study. The common symptoms were dyspnea(62%, 55 cases), cough(58%, 52 cases), recurrent respiratory tract infection(17%, 15 cases). The main CT features are reported: pleural thickening (87%, 77 cases), recurrent pneumothorax(52%, 46 cases), traction bronchiectasis(30%, 27 cases), subpleural comsolidation(20%,18 cases). All patients were proven PPFE by biopsy.34 cases received corticosteroid, 5 cases received lung transplant operation. 40 cases died during the follow?up from 4 month to 84 month. Conclusions Pleuroparenehymal fibroelastosis is a rare disease.The imaging findings were dominated by both upper lobes. Lung biopsy might be necessary. PPFE is often misdiagnosed as pulmonary tuberculosis/obsolete pulmonary tuberculosis,asbestosis,connective tissues disease and Drug?induced pneumonitis.There was no consensus on the treatment.