Department of Thoracic Surgery, The Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi, Jiangsu, China.
Department of Thoracic Surgery, The Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi, Jiangsu, China.
Transpl Immunol. 2022 Oct;74:101627. doi: 10.1016/j.trim.2022.101627. Epub 2022 May 11.
Pulmonary alveolar proteinosis (PAP) is a rarely progressive disease. This disease is characterized by the accumulation of a large amount of pulmonary surfactant in the alveolar cavity and terminal bronchiole, which is caused by the obstruction of clearance due to the weakened function of alveolar macrophages in vivo. Idiopathic PAP(IPAP) is the most common type of PAP, accounting for about 90%, and its pathogenesis remains unclear. The treatments of PAP include whole lung lavage, inhaled/subcutaneous GM-CSF, rituximab, plasmapheresis and lung transplantation. We describe a patient with IPAP who is in good condition five years after undergoing a single lung transplantation(SLT). This is the first report of IPAP treated with SLT. Accourding to the previous report and the follow-up result, lung transplantation may be an effective long-term treatment for both secondary PAP and IPAP.
肺泡蛋白沉积症（PAP）是一种罕见的进行性疾病。这种疾病的特征是肺泡腔和终末细支气管中大量肺表面活性物质的积累，这是由于体内肺泡巨噬细胞功能减弱导致清除功能受阻所致。特发性 PAP（IPAP）是 PAP 中最常见的类型，约占 90%，其发病机制仍不清楚。PAP 的治疗包括全肺灌洗、吸入/皮下 GM-CSF、利妥昔单抗、血浆置换和肺移植。我们描述了一名接受单肺移植（SLT）后五年状况良好的 IPAP 患者。这是首例接受 SLT 治疗的 IPAP 报告。根据以往的报告和随访结果，肺移植可能是治疗继发性 PAP 和 IPAP 的一种有效长期治疗方法。
