Wada Hironobu, Nakajima Takahiro, Suzuki Hidemi, Anazawa Rie, Narita Tomoharu, Terada Jiro, Yoshida Shigetoshi, Tatsumi Koichiro, Nakatani Yukio, Yoshino Ichiro
Department of General Thoracic Surgery, Chiba University Graduate School of Medicine, Chiba, Japan.
Department of Respirology, Chiba University Graduate School of Medicine, Chiba, Japan.
Gen Thorac Cardiovasc Surg. 2019 Mar;67(3):332-335. doi: 10.1007/s11748-018-0950-x. Epub 2018 May 26.
A 27-year-old female patient had presented progressing exertional dyspnea due to pulmonary hypertension. Chest CT revealed diffusely spread patchy ground-glass opacities sparing subpleural parenchymal areas suggesting the diagnosis of pulmonary veno-occlusive disease (PVOD). Despite the diagnosis of PVOD, she was somehow managed by a repetitive escalation of the epoprostenol dose and oxygen supply during the 12-month waiting period until successful bilateral lung transplantation was performed. Pathology demonstrated capillary proliferation in alveolar septae with scarce lesions of narrowed and/or occluded postcapillary small veins, leading to the final diagnosis of pulmonary capillary hemangiomatosis (PCH), not PVOD. We herein present a case of PCH diagnosed after lung transplantation with a focus on its etiology and a key to clinical diagnosis.
一名27岁女性患者因肺动脉高压出现进行性劳力性呼吸困难。胸部CT显示弥漫性分布的斑片状磨玻璃影，不累及胸膜下实质区域，提示诊断为肺静脉闭塞病（PVOD）。尽管诊断为PVOD，但在12个月的等待期内，她通过反复增加依前列醇剂量和供氧进行治疗，直到成功进行双侧肺移植。病理显示肺泡间隔毛细血管增生，肺毛细血管后小静脉狭窄和/或闭塞病变较少，最终诊断为肺毛细血管瘤病（PCH），而非PVOD。我们在此介绍一例肺移植后诊断为PCH的病例，重点关注其病因及临床诊断要点。
