Ju Y, Xu X M, Fang F, Zhang M, Li Y M
Department of Respiratory and Critical Care Medicine, Beijing Hospital, National Center of Gerontology, Beijing 100730, China.
Department of Pathology, Beijing Hospital, National Center of Gerontology, Beijing 100730, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2019 Nov 12;42(11):852-857. doi: 10.3760/cma.j.issn.1001-0939.2019.11.014.
To analyze the clinical,imaging and pathological features of Pleuroparenehymal fibroelastosis (PPFE). 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. 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. 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.
分析胸膜实质纤维弹性组织增生症（PPFE）的临床、影像学及病理特征。报道2017年4月北京医院呼吸与危重症医学科收治的1例诊断为PPFE患者的临床资料，并复习相关文献。以“胸膜实质纤维弹性组织增生症”为检索词，检索时间截至2017年10月1日，检索万方数据、中国知网及PubMed。该患者为46岁男性，表现为咳嗽、运动后气短。胸部CT扫描显示双侧不规则胸膜增厚，以上叶为主。抗结核治疗3年后病情进展。经CT引导下肺活检确诊为胸膜实质纤维弹性组织增生症（PPFE）。共报道132例（其中中文1例）。88例经病理确诊且资料详细。本研究纳入并分析了89例报道的PPFE病例的临床资料，包括48例男性和41例女性，年龄13至85岁。常见症状为呼吸困难（62%，55例）、咳嗽（58%，52例）、反复呼吸道感染（17%，15例）。报道的主要CT特征为：胸膜增厚（87%，77例）、反复气胸（52%，46例）、牵拉性支气管扩张（30%，27例）、胸膜下实变（20%，18例）。所有患者均经活检证实为PPFE。34例接受糖皮质激素治疗，5例接受肺移植手术。40例在4个月至84个月的随访期间死亡。胸膜实质纤维弹性组织增生症是一种罕见病。影像学表现以上叶为主。可能需要进行肺活检。PPFE常被误诊为肺结核/陈旧性肺结核、石棉肺、结缔组织病及药物性肺炎。治疗尚无共识。
