Studies reporting the effects of modern strategies with pulmonary arterial hypertension (PAH)-targeted therapies in sarcoidosis-associated pulmonary hypertension (S-APH) are limited. Clinical and haemodynamic data from newly diagnosed patients with severe S-APH (mean pulmonary artery pressure (mPAP) >35 mmHg or mPAP 25–35 mmHg with cardiac index <2.5 L·min−1·m−2) were collected from the French Pulmonary Hypertension Registry between 2004 and 2015. Data from 126 patients with severe S-APH were analysed (mean±sd age 57.5±11.6 years, 74% radiological stage IV). 97 patients (77%) received PAH-targeted therapy and immunosuppressive therapy was initiated or escalated in 33 patients at the time of pulmonary hypertension diagnosis. Four months after PAH-targeted therapy initiation, mean±sd pulmonary vascular resistance decreased from 9.7±4.4 to 6.9±3.0 Wood units (p<0.001), without significant improvement in exercise capacity. Among the 11 patients treated only with immunosuppressive therapy, a haemodynamic improvement was observed in four patients, including two with compressive lymph nodes. After a median follow-up of 28 months, 39 patients needed PAH-targeted therapy escalation, nine underwent lung transplantation and 42 had died. Survival at 1, 3 and 5 years was 93%, 74% and 55%, respectively. PAH-targeted therapy improved short-term pulmonary haemodynamics in severe S-APH without change in exercise capacity. Immunosuppressive therapy improved haemodynamics in selected patients. Pulmonary hypertension in sarcoidosis remains associated with a poor prognosis. Severe pulmonary hypertension remains a life-threatening complication of sarcoidosis in the modern management era http://ow.ly/fIln30etYkE

中文翻译：

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结节病相关肺动脉高压的管理和长期结果

报告现代策略与肺动脉高压 (PAH) 靶向疗法对结节病相关肺动脉高压 (S-APH) 的影响的研究是有限的。新诊断的重度 S-APH（平均肺动脉压 (mPAP) >35 mmHg 或 mPAP 25-35 mmHg，心脏指数 <2.5 L·min-1·m-2）患者的临床和血液动力学数据来自法国2004 年至 2015 年间的肺动脉高压登记。分析了 126 名重度 S-APH 患者的数据（平均±sd 年龄 57.5±11.6 岁，74% 的放射学分期为 IV）。97 名患者 (77%) 接受 PAH 靶向治疗，33 名患者在诊断肺动脉高压时开始或升级免疫抑制治疗。PAH 靶向治疗开始四个月后，平均±sd 肺血管阻力从 9.7±4.4 降至 6。9±3.0 木材单位 (p<0.001)，运动能力没有显着改善。在仅接受免疫抑制治疗的 11 名患者中，有 4 名患者的血流动力学有所改善，其中 2 名患有压迫性淋巴结。中位随访 28 个月后，39 名患者需要 PAH 靶向治疗升级，9 名患者接受肺移植，42 名患者死亡。1、3 和 5 年的存活率分别为 93%、74% 和 55%。PAH 靶向治疗在不改变运动能力的情况下改善了严重 S-APH 的短期肺血流动力学。免疫抑制治疗改善了选定患者的血流动力学。结节病的肺动脉高压仍然与预后不良有关。在现代管理时代，严重的肺动脉高压仍然是一种危及生命的结节病并发症 http://ow.