Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era.
Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era.
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DOI：
10.1136/annrheumdis-2012-202489
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发表时间：
2013-12
影响因子：
27.4
通讯作者：
Humbert M
中科院分区：
文献类型：
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作者：
Launay D;Sitbon O;Hachulla E;Mouthon L;Gressin V;Rottat L;Clerson P;Cordier JF;Simonneau G;Humbert M
评估现代管理时代新诊断的偶发性系统性硬化症（SSc）相关肺动脉高压（PAH）患者的生存率和预后因素。分析了2006年1月至2009年11月期间在法国PAH网络中入组的新诊断为PAH且无间质性肺病的Proximal SSc患者（85例患者，平均年龄64.9±12.2岁）。 PAH诊断后的中位随访时间为2.32年。 大多数患者为NYHA心功能III-IV级（79%）。PAH诊断后1年、2年和3年的总生存率分别为90%（95% CI 81%至95%）、78%（95% CI 67%至86%）和56%（95% CI 42%至68%）。年龄（HR：1.05，95%CI 1.01至1.09，p=0.012）和心脏指数（HR：0.49，95%CI 0.27至0.89，p=0.019）是单变量分析中的重要预测因素。在单变量分析中，我们还观察到性别、SSc亚型、纽约心脏协会功能分级、肺血管阻力和肺血管容量的显著趋势。相反，6分钟步行距离、平均肺动脉压和右心房压不是显著的预测因子。在多变量模型中，性别是与生存期相关的唯一独立因素（男性HR：4.76，95% CI 1.35 - 16.66，p=0.015）。即使在现代管理时代，偶发性SSC相关PAH仍然是一种毁灭性疾病。年龄、男性性别和心脏指数是该队列患者的主要预后因素。早期发现不太严重的患者应该是一个优先事项。
To assess the survival and prognostic factors in patients with newly diagnosed incident systemic sclerosis (SSc)–associated pulmonary arterial hypertension (PAH) in the modern management era. Prospectively enrolled SSc patients in the French PAH Network between January 2006 and November 2009, with newly diagnosed PAH and no interstitial lung disease, were analysed (85 patients, mean age 64.9±12.2 years). Median follow-up after PAH diagnosis was 2.32 years. A majority of patients were in NYHA functional class III–IV (79%). Overall survival was 90% (95% CI 81% to 95%), 78% (95% CI 67% to 86%) and 56% (95% CI 42% to 68%) at 1, 2 and 3 years from PAH diagnosis, respectively. Age (HR: 1.05, 95% CI 1.01 to 1.09, p=0.012) and cardiac index (HR: 0.49, 95% CI 0.27 to 0.89, p=0.019) were significant predictors in the univariate analysis. We also observed strong trends for gender, SSc subtypes, New York Heart Association functional class, pulmonary vascular resistance and capacitance to be significant predictors in the univariate analysis. Conversely, six-min walk distance, mean pulmonary arterial and right atrial pressures were not significant predictors. In the multivariate model, gender was the only independent factor associated with survival (HR: 4.76, 95% CI 1.35 to 16.66, p=0.015 for male gender). Incident SSc-associated PAH remains a devastating disease even in the modern management era. Age, male gender and cardiac index were the main prognosis factors in this cohort of patients. Early detection of less severe patients should be a priority.
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影响因子：
--
作者：
Guenther, S.;Jais, X.;Montani, D.
通讯作者：
Montani, D.
影响因子：
24.3
作者：
Mathai, S. C.;Girgis, R. E.;Hassoun, P. M.
通讯作者：
Hassoun, P. M.
影响因子：
24.3
作者：
Mathai, S. C.;Bueso, M.;Hassoun, P. M.
通讯作者：
Hassoun, P. M.
影响因子：
27.4
作者：
Fransen, J.;Popa-Diaconu, D.;Vonk, M. C.
通讯作者：
Vonk, M. C.
影响因子：
27.4
作者：
Hachulla AL;Launay D;Gaxotte V;de Groote P;Lamblin N;Devos P;Hatron PY;Beregi JP;Hachulla E
通讯作者：
Hachulla E