Arndt Patrick
Division of Pulmonary, Allergy, Critical Care, and Sleep Medicine, Department of Medicine, University of Minnesota, Minneapolis, MN 55455, USA.
Biomedicines. 2023 Mar 16;11(3):924. doi: 10.3390/biomedicines11030924.
Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that is currently classified as an idiopathic interstitial pneumonia. Although originally described as an idiopathic disease, PPFE has now been identified as a rare complication following hematopoietic stem cell transplant (HSCT). Unlike other pulmonary complications after HSCT, PPFE occurs very late after transplant. Etiologies for PPFE after HSCT remain to be fully established. Infections and adverse effects to alkylating chemotherapy have been suggested as possible causes. In several cases, there is an association of PPFE with bronchiolitis obliterans syndrome after HSCT, suggesting that PPFE may be another manifestation of pulmonary chronic graft versus host disease after HSCT. Algorithms have been designed to assist in confirming a diagnosis of PPFE without the need for a surgical lung biopsy, however at present, no biomarker is established for the diagnosis or to predict the progression of disease. Presently, there is no current therapy for PPFE, but fortunately the disease progresses slowly in most patients.
胸膜实质纤维弹性组织增生症（PPFE）是一种罕见疾病，目前被归类为特发性间质性肺炎。尽管最初被描述为一种特发性疾病，但PPFE现在已被确定为造血干细胞移植（HSCT）后的一种罕见并发症。与HSCT后的其他肺部并发症不同，PPFE在移植后很晚才出现。HSCT后PPFE的病因仍有待充分明确。感染和对烷化剂化疗的不良反应被认为是可能的原因。在一些病例中，PPFE与HSCT后的闭塞性细支气管炎综合征有关，这表明PPFE可能是HSCT后肺部慢性移植物抗宿主病的另一种表现形式。已经设计了一些算法来协助在无需手术肺活检的情况下确诊PPFE，然而目前，尚未建立用于诊断或预测疾病进展的生物标志物。目前，尚无针对PPFE的现有治疗方法，但幸运的是，大多数患者的病情进展缓慢。
