大动脉炎相关肺动脉高压临床特点和预后

Clinical features and outcome of Takayasu arteritis associated pulmonary hypertension

目的分析大动脉炎相关肺动脉高压（Takayasu arteritis associated pulmonaryhy pertension，TA-PH）临床表现、诊治及预后。方法回顾性分析2008年至2017年上海市肺科医院确诊的13例TA．PH患者，排除其他类型PH。收集患者基线资料，包括血流动力学参数，并进行随访。采用均数±标准差（i坛）或百分率描述基线，采用Kaplan-Meier法计算生存率。结果患者年龄（39±11）岁，女性10例。首发症状至确诊2个月至50年，10例TA和PH同时诊断。最常见表现为活动后气促（12例），胸闷胸痛（8例）和心悸（6例），WHO功能分级Ⅱ-Ⅲ级，肺平均动脉压（48．0±14．0）mmHg和肺血管阻力（7．59±4．21）WoodU显著升高。8例在TA活动期。CT肺血管造影和肺动脉造影示血管壁增厚，管腔狭窄、闭塞或扩张。所有患者均用PH靶向药物，部分联合糖皮质激素。4例行肺动脉内支架植入术。3例死亡。结论TA有发生PH风险，且可能以PH为首发表现。TA合并活动后气促建议常规筛查PH。TA合并PH预后差。PH靶向药物和血管重建可有效治疗TA-PH，解除肺血管狭窄，但确切疗效仍需大样本研究验证。

Objective To investigate the clinical features, diagnosis, treatment and outcome of patients with Takayasu arteritis associated pulmonary hypertension （TA-PH）. Methods Patients diagnosed as TA-PH in Shanghai Pulmonary hospital from 2008 to 2017 were retrospectively reviewed and followed up. Baseline characteristics including hemodynamics were collected. Data were summarized as mean ± standard deviation or frequency （%）. Survival analyses were performed using the Kaplan- Meier method. Results Thirteen TA-PH patients （10 female, aged 39±11 years old） were included. The duration from symptoms onset to diagnosis was 2 months to 50 years, and ten patients were diagnosed TA and PH at the same time. Shortness of breath was the most common clinical manifestation （12 cases）, followed by chest pain and tightness （8 cases） and palpitation （6 cases）. All patients had a moderated WHO functional class and 8 patients were in active phase. Vessel wall thickening, lumen narrowing, occlusion and/or dilation were found in CT pulmonary angiography and angiography. Mean pulmonary arterial pressure （48.0±14.0） mmHg and pulmonary vascular resistant （7.59±4.21） Wood U were increased. All patients received PH-specific therapies, and patients at active status took glucocorticoid. Stent implantation in pulmonary artery was performed in 4 patients. Three patients died during the follow- up. Conclusions Patients with TA are at risk of PH, and PH can be the first manifestation of TA, which suggest that PH should be screened regularly in patients with TA and shortness of breath. The prognosis of TA-PH is poor. PH-specific therapies and vascular reconstruction therapy may be effective, but need further investigation.