Gui X H, Qiu Y Y, Chen T T, Li H, Dai J H, Cai H R, Xiao Y L, Cao M
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.
Zhonghua Jie He He Hu Xi Za Zhi. 2022 May 12;45(5):453-459. doi: 10.3760/cma.j.cn112147-20210917-00655.
To raise the awareness of idiopathic pleuroparenehymal fibroelastosis (iPPFE) through investigating the clinical, radiographic and pathological features. Five cases of iPPFE proved by pathology. The clinical data were studied respectively, and the relevant literature was reviewed. 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. 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.
通过研究特发性胸膜肺实质纤维弹性组织增生症（iPPFE）的临床、影像学和病理特征，提高对该疾病的认识。5例经病理证实的iPPFE患者。分别对其临床资料进行研究，并复习相关文献。所有iPPFE病例均表现为咳嗽和呼吸困难。患者包括3名男性和2名女性，年龄在30至70岁之间。胸部CT扫描显示胸膜增厚，双上肺胸膜下实变并伴有牵拉性支气管扩张。进行了计算机断层扫描引导下经皮肺活检或手术肺活检，相同病理显示胸膜和胸膜下有致密的弹性纤维和胶原纤维。弹性纤维染色也呈阳性，符合PPFE。1例患者接受低剂量皮质类固醇治疗，2例接受吡非尼酮治疗，其余未接受治疗。3例患者在随访期间病情稳定。iPPFE具有特征性病理表现。然而，由于漏诊或误诊，临床报道病例数较少。提高对iPPFE特征的认识有助于该病的诊断、治疗及预后判断。
