Huang Hui, Feng Ruie, Li Shan, Wu Bo, Xu Kai, Xu Zuojun, Chen Jingyu
Department of Respiratory Medicine Pathological Department, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing Department of Cardiothoracic Surgery, Lung transplant Group, Wuxi People's Hospital, Nanjing Medical University, Wuxi, Jiang Su Radiological Department, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Medicine (Baltimore). 2017 May;96(19):e6900. doi: 10.1097/MD.0000000000006900.
Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease that is characterized radiologically by apical pleural thickening and histologically by elastic fibrosis of the visceral pleura. Although PPFE cases have been reported occasionally since this disease was initially described, most such cases have involved secondary PPFE. Idiopathic PPFE (iPPFE) cases have been less thoroughly studied. There are no effective medications for patients with iPPFE.
A 34-year-old man with no asbestos or cigarette exposure was admitted to our ward due to worsening cough and exertional dyspnea for 10 years. He had a "flattened thoracic cage" and bibasilar inspiratory crackles without finger clubbing. A series of chest computed tomography scans during the preceding 10 years revealed the presence of gradual, exaggerated, upper lung-predominant, diffuse pleural thickening and dense subpleural opacification with traction bronchiectasis.
He was performed with video-assisted thoracic surgical (VATS) lung biopsy. The pulmonary histopathologic examination showed thickened visceral pleura and prominent subpleural fibroelastosis, confirming the diagnosis of iPPFE.
After the failure of treatment with prednisone plus cyclophosphamide and sequential pirfenidone administration, he was arranged with bilateral lung transplantation two years later.
The patient did not require supplemental oxygenation anymore after he recovered from lung transplantation.
Bilateral lung transplantation might be tried for the end-stage iPPFE cases.
胸膜实质纤维弹性组织增生症（PPFE）是一种罕见的间质性肺病，其放射学特征为肺尖胸膜增厚，组织学特征为脏层胸膜弹性纤维化。尽管自该病首次被描述以来偶尔有PPFE病例报道，但大多数此类病例为继发性PPFE。特发性PPFE（iPPFE）病例的研究较少。对于iPPFE患者尚无有效的药物治疗。
一名34岁男性，无石棉或吸烟接触史，因咳嗽加重和劳力性呼吸困难10年入院。他有“胸廓扁平”和双肺底吸气性啰音，无杵状指。此前10年的一系列胸部计算机断层扫描显示存在逐渐加重、以肺上叶为主的弥漫性胸膜增厚和致密的胸膜下混浊，并伴有牵拉性支气管扩张。
对他进行了电视辅助胸腔镜手术（VATS）肺活检。肺组织病理学检查显示脏层胸膜增厚和明显的胸膜下纤维弹性组织增生，确诊为iPPFE。
在泼尼松加环磷酰胺治疗失败及随后使用吡非尼酮治疗后，两年后为他安排了双侧肺移植。
该患者肺移植康复后不再需要吸氧。
对于终末期iPPFE病例可尝试双侧肺移植。
