肺静脉闭塞病/肺毛细血管瘤病的临床特征及诊治进展  

Clinical Features,Diagnosis and Treatment of Pulmonary Veno-Occlusive Disease/Pulmonary Capillary Hemangiomatosis

作　　者：胡慧 谢军平[1] HU Hui;XIE Jun-ping(Department of Respiratory and Critical Care Medicine,the Second Affiliated Hospital of Nanchang University,Nanchang 330006,China)

机构地区：[1]南昌大学第二附属医院呼吸与危重症医学科,南昌330006

出　　处：《南昌大学学报（医学版）》2022年第3期73-76,83,共5页Journal of Nanchang University：Medical Sciences

基　　金：国家自然科学地区科学基金(81960425);江西省卫生与计划生育委员会中医药课题(2014B041);江西省科技厅重点研发计划(20181BBH80005)。

摘　　要：肺毛细血管瘤病(pulmonary angiomatosis,PCH)是一种罕见的肺动脉高压(pulmonary arterial hypertension,PAH),其特征是肺泡间隔毛细血管增生。肺静脉闭塞病(pulmonary veno-occlusive disease,PVOD)组织学表现与PCH有明显的重叠,被认为是同一种疾病的不同表现,归属于PCH。基因检测可使PVOD/PCH更早得到确诊,但其病程进展快,治疗无循证医学证据,预后也更差,靶向治疗部分可能导致危及生命的肺水肿,肺移植仍然是符合条件的患者唯一首选治疗方法。进一步了解PVOD/PCH的分子机制和探索新的治疗靶点是今后的研究方向。Pulmonary capillary hemangiomatosis(PCH)is a rare form of pulmonary arterial hypertension(PAH)that is characterized by pulmonary septal capillary hyperplasia.Pulmonary veno-occlusive disease(PVOD)has obvious histological overlap with PCH.It is considered to be a different manifestation of PCH and to belong to PCH.Genetic testing can lead to an earlier diagnosis of PVOD/PCH.However,the disease progresses rapidly,and the treatment is not evidence-based and is associated with a poor prognosis.Furthermore,the targeted therapy may partially result in life-threatening pulmonary edema.Therefore,lung transplantation remains the only treatment of choice for eligible patients.Further understanding of the molecular mechanism of PVOD/PCH and exploring new therapeutic targets will be the future research direction.

关 键 词：肺毛细血管瘤 肺静脉闭塞病 肺动脉高压 临床特征 诊断 治疗 编码真核翻译起始因子2激酶4基因 

分 类 号：R543.2[医药卫生—心血管疾病]