肺淋巴管肌瘤病相关肺动脉高压一例

Pulmonary Hypertension in Pulmonary Lymphangiomyomatosis:a Case Report

肺淋巴管肌瘤病(LAM)是一种罕见的主要发生于育龄期女性的慢性进展性弥漫性囊性肺疾病,肺动脉高压是LAM的一种少见并发症,目前LAM相关肺动脉高压在流行病学、发病机制、治疗策略等方面证据匮乏。本文报道一例表现为气短的育龄期女性,结合特异性肺部影像学表现及血清血管内皮生长因子-D诊断为LAM,经右心导管检查证实为毛细血管前性肺动脉高压,经西罗莫司治疗及家庭氧疗病情稳定。

Pulmonary lymphangiomyomatosis(LAM)is a rare chronic progressive diffuse cystic lung disease that mainly occurs in women of reproductive age.Pulmonary hypertension is a rare complication of LAM.Currently,there is insufficient evidence on the epidemiology,pathogenesis and treatment strategy of LAM related pulmonary hypertension.We reported a case of a woman at reproductive age with shortness of breath and diagnosed with LAM by the combination of specific lung imaging features and serum vascular endothelial growth factor D.Precapillary pulmonary hypertension was confirmed by right cardiac catheterization.Her condition was stable with Sirolimus and home oxygen therapy.