Department of Epidemiology & Preventive Medicine, Monash University, Victoria, Australia.
Int J Rheum Dis. 2009 Sep;12(3):192-206. doi: 10.1111/j.1756-185X.2009.01410.x.
Fibrosis, inflammation and vascular dysfunction are major features of systemic sclerosis and the multiple organ-specific complications that characterize this disease. Several manifestations of systemic sclerosis, including Raynaud's phenomenon, digital ulceration, scleroderma renal crisis and pulmonary arterial hypertension, contribute significantly to morbidity and mortality and are understood to share similarities in their underlying vasculopathy. In recent years, a number of treatment options have become available that ease the burden of these manifestations, including calcium channel blockers, angiotensin converting enzyme inhibitors, prostanoids and prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase type-5 inhibitors. Several of these treatments have demonstrated beneficial effects against more than one complication, as a result of the similarities in the pathology underlying these manifestations. However, physicians involved in the management of patients with systemic sclerosis are faced with differing levels of evidence supporting these treatments, and historically little international consensus on the treatment of some manifestations, such as digital ulcers. The aim of this article is to evaluate the level of evidence supporting each intervention in systemic sclerosis, thereby facilitating decision-making in the clinic.
纤维化、炎症和血管功能障碍是全身性硬皮病的主要特征，也是导致该病出现多种器官特异性并发症的原因。全身性硬皮病的几种表现，包括雷诺现象、指端溃疡、硬皮病肾危象和肺动脉高压，极大地影响了发病率和死亡率，并且被认为在其潜在血管病变方面具有相似性。近年来，出现了许多缓解这些表现的治疗选择，包括钙通道阻滞剂、血管紧张素转换酶抑制剂、前列腺素和前列环素类似物、内皮素受体拮抗剂和磷酸二酯酶 5 抑制剂。其中一些治疗方法针对多种并发症显示出有益的效果，这是由于这些表现的病理学基础存在相似性。然而，参与系统性硬皮病患者管理的医生面临着支持这些治疗方法的证据水平不同的情况，而且在历史上，对于某些表现（如指端溃疡）的治疗几乎没有国际共识。本文的目的是评估每种干预措施在系统性硬皮病中的证据水平，从而为临床决策提供便利。
