García Hernández Francisco José, Castillo Palma María Jesús, Montero Mateos Enrique, González León Rocío, López Haldón José Eduardo, Sánchez Román Julio
Servivio de Medicina Interna, Hospital Universitario Virgen del Rocío, Sevilla, España.
Servivio de Medicina Interna, Hospital Universitario Virgen del Rocío, Sevilla, España.
Med Clin (Barc). 2016 Jan 1;146(1):1-7. doi: 10.1016/j.medcli.2015.04.029. Epub 2015 Jul 10.
Pulmonary arterial hypertension (PAH) is an important cause of morbimortality in systemic sclerosis (SSc). Evolution is worse than that of subjects with idiopathic PAH, but prognosis improves when PAH is diagnosed early. The aim of this research is to describe results of a screening program for diagnosis of pulmonary hypertension (PH) carried out in a cohort of Spanish patients with SSc.
PH screening was performed by transthoracic doppler echocardiography (TTDE) in 184 patients with SSc. Patients with systolic pulmonary arterial pressure estimated by TTDE>35 mmHg were evaluated per protocol to confirm diagnosis and type of PH.
PAH was diagnosed in 25 patients (13.6%). Patients with diffuse and limited SSc developed PAH in a similar degree, 9/60 (15%) vs. 16/100 (16%), with no cases among patients with SSc "sine scleroderma" or "pre-scleroderma" (P<.001). The only clinical or epidemiological data characterizing patients with PAH were older age (mean age 67 years for patients with PAH vs. 56 years for those without PAH, P=.007), limited SSc, a trend toward shorter evolution of the underlying disease (median 8 years for patients with PAH vs. 10 years for those without PAH, P=.73), and a higher frequency of positive anticentromere antibodies (16 patients [64%] with PAH vs. 70 (48,3%) without PAH, P=.19).
Prevalence of PAH in SSc was high and supports the implementation of a regular screening program.
肺动脉高压（PAH）是系统性硬化症（SSc）患者发病和死亡的重要原因。其病情进展比特发性PAH患者更严重，但早期诊断PAH可改善预后。本研究旨在描述在一组西班牙SSc患者中开展的肺动脉高压（PH）诊断筛查项目的结果。
对184例SSc患者进行经胸多普勒超声心动图（TTDE）检查以筛查PH。根据方案对TTDE估计收缩期肺动脉压>35 mmHg的患者进行评估，以确诊PH及其类型。
25例患者（13.6%）被诊断为PAH。弥漫性和局限性SSc患者发生PAH的程度相似，分别为9/60（15%）和16/100（16%），而“无硬皮病”或“硬皮病前期”的SSc患者中未出现PAH病例（P<0.001）。PAH患者唯一具有特征性的临床或流行病学数据是年龄较大（PAH患者平均年龄67岁，无PAH患者平均年龄56岁，P = 0.007）、局限性SSc、基础疾病病程有缩短趋势（PAH患者中位数为8年，无PAH患者为10年，P = 0.73）以及抗着丝点抗体阳性率较高（PAH患者中有16例[64%]，无PAH患者中有70例[48.3%]，P = 0.19）。
SSc患者中PAH的患病率较高，支持实施定期筛查项目。
