Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
Front Med. 2019 Apr;13(2):259-266. doi: 10.1007/s11684-018-0634-z. Epub 2018 Apr 19.
Lymphangioleiomyomatosis (LAM) is a rare diffuse cystic lung disease. Knowledge on LAM-related pulmonary hypertension (PH) is limited. This study aimed to analyze the clinical characteristics of LAM with elevated pulmonary artery pressure (PAP) and evaluate the potential efficacy of sirolimus. The study involved 50 LAM patients who underwent echocardiography. According to the tricuspid regurgitation velocity (TRV), these patients were divided into the TRV ⩽ 2.8 m/s group and TRV > 2.8 m/s group. Both groups comprised 25 females with an average age of 38.6 ± 8.1 and 41.5 ± 8.9 years. In the TRV > 2.8 m/s group, the estimated systolic PAP (SPAP) was significantly elevated (52.08 ± 12.45 mmHg vs. 30.24 ± 5.25 mmHg, P < 0.01). Linear analysis showed that SPAP was correlated with forced expiratory volume in 1 s (FEV), diffusing capacity of the lungs for carbon monoxide, alveolar arterial oxygen gradient (PO), and 6 min walking distance (r =-0.392, -0.351, 0.450, and -0.591, respectively; P < 0.05), in which PO was a risk factor for SPAP elevation (β = 0.064, OR = 1.066, P < 0.05). Moreover, in 10 patients who received sirolimus therapy, SPAP decreased from 57.0 12.6 mmHg to 35.2 ± 11.1 mmHg. The study showed that LAM patients with PH exhibit poor pulmonary function and hypoxemia and may benefit from sirolimus treatment.
淋巴管平滑肌瘤病（LAM）是一种罕见的弥漫性囊性肺疾病。关于 LAM 相关肺动脉高压（PH）的知识有限。本研究旨在分析肺动脉压升高（PAP）的 LAM 患者的临床特征，并评估西罗莫司的潜在疗效。该研究纳入了 50 例接受超声心动图检查的 LAM 患者。根据三尖瓣反流速度（TRV），将这些患者分为 TRV ⩽ 2.8 m/s 组和 TRV > 2.8 m/s 组。两组均包含 25 名女性，年龄分别为 38.6 ± 8.1 岁和 41.5 ± 8.9 岁。在 TRV > 2.8 m/s 组中，估计的收缩期 PAP（SPAP）明显升高（52.08 ± 12.45 mmHg 比 30.24 ± 5.25 mmHg，P < 0.01）。线性分析显示，SPAP 与用力呼气量（FEV）、一氧化碳弥散量、肺泡动脉氧梯度（PO）和 6 分钟步行距离呈负相关（r =-0.392、-0.351、0.450 和-0.591，分别；P < 0.05），其中 PO 是 SPAP 升高的危险因素（β = 0.064，OR = 1.066，P < 0.05）。此外，在接受西罗莫司治疗的 10 例患者中，SPAP 从 57.0 ± 12.6 mmHg 降至 35.2 ± 11.1 mmHg。研究表明，PH 的 LAM 患者肺功能差，伴有低氧血症，可能受益于西罗莫司治疗。
