期刊
EUROPEAN RESPIRATORY JOURNAL
卷 50, 期 4, 页码 -出版社
EUROPEAN RESPIRATORY SOC JOURNALS LTD
DOI: 10.1183/13993003.00465-2017
关键词
-
资金
- Departement Hospitalo-Universitaire Thorax Innovation (TORINO)
- Laboratoire d'Excellence en Recherche sur le Medicament et l'Innovation Therapeutique (LERMIT)
- Projet Hospitalier de Recherche Clinique (PHRC) HYpertension pulmonaire des Pneumopathies Interstitielles Diffuses (HYPID)
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.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据