Arthritis Center, Boston University School of Medicine, Boston, Massachusetts, USA.
Curr Opin Rheumatol. 2011 Nov;23(6):536-44. doi: 10.1097/BOR.0b013e32834ba6a7.
The review provides an update of the epidemiology, pathogenesis, risk factors, screening and treatment of pulmonary arterial hypertension in systemic sclerosis.
Several recent studies have investigated the utility of several noninvasive screening methods and the propagation of new treatments promise the clinician better outcomes than the current median survival time of 1 year for patients with scleroderma-related pulmonary arterial hypertension.
Pulmonary hypertension is a frequent cause of morbidity and mortality in patients with systemic sclerosis. This review discusses the recent changes in the classification of pulmonary hypertension, especially the significance for the rheumatologist. A high clinical suspicion should be maintained, even in early scleroderma. Despite progress in echocardiography and biomarkers, right heart catheterization remains the only test that can diagnose pulmonary hypertension and differentiate pulmonary veno-occlusive disease from pulmonary arterial hypertension. The differentiation of these causes of pulmonary hypertension in the scleroderma patient is essential because the initiation of pulmonary vasodilators in veno-occlusive disease often leads to increased mortality. The role of screening with serum biomarkers and noninvasive testing remains controversial, and in this review we discuss the controversies and new recommendations in detail.
本文综述了系统性硬化症相关肺动脉高压的流行病学、发病机制、危险因素、筛查和治疗的最新进展。
多项近期研究调查了多种非侵入性筛查方法的效用，新疗法的出现有望为临床医生带来更好的治疗效果，改善患者预后。目前，硬皮病相关肺动脉高压患者的中位生存时间仅为 1 年。
肺动脉高压是系统性硬化症患者发病率和死亡率的主要原因。本文讨论了肺动脉高压分类的最新变化，特别是对风湿病学家的意义。即使在早期硬皮病患者中，也应保持高度的临床警惕性。尽管超声心动图和生物标志物取得了进展，但右心导管检查仍然是诊断肺动脉高压并区分肺静脉闭塞性疾病与肺动脉高压的唯一方法。区分硬皮病患者的这些肺动脉高压病因至关重要，因为在静脉闭塞性疾病中使用肺动脉扩张剂通常会导致死亡率增加。使用血清生物标志物和非侵入性检测进行筛查的作用仍存在争议，本文详细讨论了这些争议和新建议。
