纤维性纵隔炎致肺动脉狭窄研究现状  

Pulmonary Artery Stenosis Caused by Fibrosing Mediastinitis

作　　者：孙策 卢志雄 汤银江 SUN Ce;LU Zhixiong;TANG Yinjiang(Department of Cardiovascular Medicine,Northeast Yunnan Regional Central Hospital,Zhaotong 657000,Yunnan,China;Department of Respiratory and Pulmonary Vascular Diseases,Fuwai Cardiovascular Disease Hospital of Yunnan,Chinese Academy of Medical Sciences,Kunming 650000,Yunnan,China)

机构地区：[1]云南省滇东北区域中心医院心血管内科,云南昭通657000 [2]云南省阜外心血管病医院呼吸与肺血管科,云南昆明650000

出　　处：《心血管病学进展》2024年第1期24-26,共3页Advances in Cardiovascular Diseases

摘　　要：纤维性纵隔炎(FM)又称为硬化性纵隔炎或纵隔纤维化,是以纵隔内纤维组织良性增生为特点的一类罕见疾病。随纵隔内纤维组织增生,血管、气管及食管受压后逐渐出现相应压迫症状。该疾病进展早期可无明显临床表现,易漏诊。因FM较为罕见,病例样本量小,故目前临床研究有限,病因及发病机制尚不明确且无规范诊疗指南。现就FM致肺动脉狭窄研究现状做阐述,旨在对FM相关性肺动脉狭窄诊疗提供指导意义。Fibrosing mediastinitis(FM)is also known as sclerosing mediastinitis or mediastinal fibrosis,it is a rare disease characterized by benign hyperplasia of mediastinal fibrous tissue.With the proliferation of fibrous tissue in the mediastinum,corresponding compression symptoms gradually appear after compression of blood vessels,trachea and esophagus.In the early stage of disease progression,there may be no clinical manifestations obviously,and it is easy to misdiagnose.Due to the rarity of FM and the small sample size of cases,the clinical research is limited,the etiology and pathogenesis are still unclear,and there is no standardized diagnosis and treatment guideline of FM.This article expounds the research status of pulmonary artery stenosis caused by FM,aiming to provide guidance for the diagnosis and treatment of pulmonary artery stenosis associated with FM.

关 键 词：纤维性纵隔炎 纤维组织增生 肺动脉狭窄 诊疗 

分 类 号：R564.2[医药卫生—呼吸系统] R543.2[医药卫生—内科学]