Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis

作者：Yidan Li, Xiangli Meng, Yidan Wang, Yuanhua Yang, Xiuzhang Lu · 发表于：Medicine · 年份：2018 · DOI：10.1097/md.0000000000009694 · 被引用次数：16 · 研究领域：IgG4-Related and Inflammatory Diseases、Pneumothorax, Barotrauma, Emphysema、Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

INTRODUCTION: Fibrosingmediastinitis (FM) is caused by a proliferation of fibrous tissue in the mediastinum encasing the mediastinal viscera that results in compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of FM caused by extrinsic compression of pulmonary blood vessels. CASE PRESENTATION: Here, we present the case of a 47-year-old man who presented with a 10-year history of progressive hemoptysis and a 2-year history of shortness of breath, in whom a diagnosis of FM was made. Occlusion of the superior pulmonary veins was noted, with stenosis of the inferior pulmonary veins, leading to PH. Because the patient was a poor candidate for interventional catheterization, the preferred treatment for FM, his PH has been managed with diuretics, and he remains stable. CONCLUSIONS: FM is a serious, potentially life-threatening condition that is best managed in specialized centers.