Matos Harold, Maskey Ashish, Keshavamurthy Suresh, Miller Jordan, Nandavaram Sravanthi
Department of Medicine, University of Kentucky, Lexington, Kentucky.
Department of Surgery, University of Kentucky, Lexington, Kentucky.
JHLT Open. 2023 Aug 6;1:100002. doi: 10.1016/j.jhlto.2023.100002. eCollection 2023 Oct.
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder caused by defective granulocyte-macrophage colony-stimulating factor signaling, leading to abnormalities of macrophage metabolic and immune functions, with resultant impaired surfactant metabolism and its accumulation within the alveoli. PAP can relapse in patients who underwent lung transplantation for PAP related to genetic defects. However, its occurrence is exceedingly rare in lung allografts of patients who underwent lung transplantation for other primary end-stage lung disease. Prompt diagnosis and appropriate treatment strategy are crucial to prevent the decline in the allograft function and to avoid unnecessary empiric treatments for rejection and/ infection. Here we present a case of PAP in a patient, 8 months postbilateral lung transplantation for COVID-19 fibrosis.
肺泡蛋白沉积症(PAP)是一种罕见的肺部疾病,由粒细胞-巨噬细胞集落刺激因子信号缺陷引起,导致巨噬细胞代谢和免疫功能异常,进而导致表面活性物质代谢受损并在肺泡内积聚。对于因基因缺陷相关的PAP而接受肺移植的患者,PAP可能会复发。然而,在因其他原发性终末期肺病而接受肺移植的患者的肺同种异体移植中,其发生极为罕见。及时诊断和适当的治疗策略对于防止同种异体移植功能下降以及避免针对排斥反应和/或感染进行不必要的经验性治疗至关重要。在此,我们报告一例在因新冠病毒肺炎纤维化接受双侧肺移植8个月后的患者中发生PAP的病例。