Cha Yoon Jin, Han Joungho, Chung Man Pyo, Kim Tae Jung, Shin Sumin
Department of Pathology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Clin Respir J. 2018 Apr;12(4):1495-1502. doi: 10.1111/crj.12696. Epub 2017 Sep 7.
Pleuroparenchymal fibroelastosis (PPFE) is classified as a rare idiopathic interstitial pneumonia, and its clinicopathologic features remain unclear. We sought to investigate the clinical, radiologic, and pathologic features of patients with histologically proven PPFE.
Seven patients who had histologic features of PPFE that were definitive or consistent with PPFE were included. Clinical data and radiologic findings of chest computed tomography were obtained. Histologic evaluation was performed by 2 pathologists.
Four patients had elevated serum autoantibodies, 3 had a history of organ transplantation, and 1 had acute Aspergillus infection. All patients had various degrees of restrictive patterns on pulmonary function tests. Histologic examination revealed a variable extent of PPFE. Fibroblastic foci, patchy lymphocytic infiltration, and pneumothorax were observed, but these findings were nonspecific. Radiologically, PPFE lesions were distributed diffusely, rather than being limited to the upper lungs.
PPFE seems to be a histologic finding that may partly overlap with other chronic lung diseases. There can be various underlying clinical states.
胸膜实质纤维弹性组织增生症（PPFE）被归类为一种罕见的特发性间质性肺炎，其临床病理特征仍不明确。我们旨在研究经组织学证实的PPFE患者的临床、放射学和病理特征。
纳入7例具有明确或符合PPFE组织学特征的患者。获取临床资料和胸部计算机断层扫描的放射学表现。由2名病理学家进行组织学评估。
4例患者血清自身抗体升高，3例有器官移植史，1例有急性曲霉菌感染。所有患者在肺功能测试中均有不同程度的限制性模式。组织学检查显示PPFE程度各异。观察到成纤维细胞灶、散在淋巴细胞浸润和气胸，但这些表现无特异性。放射学上，PPFE病变弥漫分布，而非局限于上肺。
PPFE似乎是一种组织学表现，可能部分与其他慢性肺病重叠。可能存在多种潜在临床状态。
