Abstract:
Pulmonary alveolar proteinosis (PAP) affecting transplanted lungs is not well recognized. Herein, we report two cases of PAP after lung transplantation (LTx). The first case was a 4-year-old boy with hereditary pulmonary fibrosis who underwent bilateral LTx and presented with respiratory distress on postoperative day (POD) 23. He was initially treated for acute rejection, died due to infection on POD 248, and was diagnosed with PAP at autopsy. The second case involved a 52-year-old man with idiopathic pulmonary fibrosis who underwent bilateral LTx. On POD 99, chest computed tomography revealed ground-glass opacities. Bronchoalveolar lavage and transbronchial biopsy led to a diagnosis of PAP. Follow-up with immunosuppression tapering resulted in clinical and radiological improvement. PAP after lung transplantation mimics common acute rejection; however, is potentially transient or resolved with tapering immunosuppression, as observed in the second case. Transplant physicians should be aware of this rare complication to avoid misconducting immunosuppressive management.
摘要:
影响移植肺的肺泡蛋白沉积症(PAP)尚未得到很好的认识。在这里,我们报告了2例肺移植(LTx)后PAP。第一例是一名遗传性肺纤维化的4岁男孩,他接受了双侧LTx治疗,并在术后第23天(POD)出现呼吸窘迫。他最初因急性排斥而接受治疗,由于感染POD248而死亡,并在尸检中被诊断为PAP。第二例涉及一名患有特发性肺纤维化的52岁男子,他接受了双侧LTx。在POD99上,胸部计算机断层扫描显示毛玻璃混浊。支气管肺泡灌洗和经支气管活检可诊断为PAP。免疫抑制逐渐减少的后续行动导致临床和放射学改善。肺移植后的PAP模拟常见的急性排斥反应;然而,可能是短暂的,或者通过逐渐减弱的免疫抑制来解决,正如在第二种情况下观察到的那样。移植医师应意识到这种罕见的并发症,以避免进行免疫抑制管理。