Diagnosis and management of pulmonary hypertension in systemic sclerosis.
Diagnosis and management of pulmonary hypertension in systemic sclerosis.
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DOI：
10.1007/s11926-009-0078-1
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发表时间：
2010-02-01
影响因子：
5
通讯作者：
Archer, Stephen L
中科院分区：
文献类型：
--
作者：
Sweiss, Nadera J;Hushaw, Linda;Archer, Stephen L
系统性硬化症 (SSc) 患者可能会出现由肺动脉高压 (PAH)、左心室疾病或肺纤维化引起的肺动脉高压 (PH；平均肺动脉压 >/= 25 mm Hg)。 PAH是一种肺血管疾病，其诊断需要肺毛细血管楔压小于15毫米汞柱，肺血管阻力大于3伍德单位，并排除血栓栓塞和实质肺疾病。 PAH-SSc 的分子机制包括血管系统和右心室中炎症和纤维化途径的激活。循环自身抗体触发内皮损伤和成纤维细胞激活。 PAH 最常见于患有局限性皮肤病和抗着丝粒抗体的患者的晚期并发症。尽管超声心动图是一种有用的筛查工具，但在开始治疗之前需要进行心导管检查来诊断 PAH。 PAH-SSc 的预后和治疗反应比其他 PAH 类别更差（中位生存期，1-3 年）。批准的疗法包括前列环素、内皮素拮抗剂和 5 型磷酸二酯酶抑制剂。需要进行研究来确定疾病机制并开发有效的疗法。
Patients with systemic sclerosis (SSc) can develop pulmonary hypertension (PH; mean pulmonary artery pressure >/= 25 mm Hg) caused by pulmonary arterial hypertension (PAH), left ventricular disease, or pulmonary fibrosis. PAH is a pulmonary vascular disease, the diagnosis of which requires pulmonary capillary wedge pressure less than 15 mm Hg, pulmonary vascular resistance greater than 3 Wood Units, and exclusion of thromboembolism and parenchymal lung disease. Molecular mechanisms underlying PAH-SSc include activation of inflammatory and fibrogenic pathways in the vasculature and right ventricle. Circulating autoantibodies trigger endothelial damage and fibroblast activation. PAH most commonly occurs as a late complication in patients with limited cutaneous disease and anticentromere antibodies. Although echocardiography is a useful screening tool, heart catheterization is required to diagnose PAH before initiating therapy. Prognosis and therapeutic response are worse in PAH-SSc than in other PAH categories (median survival, 1-3 y). Approved therapies include prostacyclins, endothelin antagonists, and phosphodiesterase type 5 inhibitors. Research is needed to define disease mechanisms and develop effective therapies.