Shetty Nagalakshmi, Derk Chris T
Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA 19107, USA.
Inflamm Allergy Drug Targets. 2011 Feb;10(1):19-26. doi: 10.2174/187152811794352088.
Systemic sclerosis (SSc) is a multisystem connective tissue disease of unknown etiology that is characterized by inflammation, vascular dysfunction and fibrosis of the skin and visceral organs. SSc is clinically diverse both in terms of the burden of skin and organ involvement and the rate of progression of the disease. Recent studies indicate that the endothelin system, especially ET-1 and the ETA and ETB receptors may play a key role in the pathogenesis of SSc. A new class of drugs, endothelin receptor antagonists has been introduced for treatment of patients with pulmonary arterial hypertension (PAH). Bosentan, a dual endothelin receptor antagonist as well as Sitaxsentan and Ambrisentan, selective blockers of the ETA receptor have proven effective in SSc-PAH. This effect may be mediated through both a vasodilatory and antifibrotic effect, thus making these agents attractive as potential disease modifying agents for SSc.
系统性硬化症（SSc）是一种病因不明的多系统结缔组织疾病，其特征为皮肤和内脏器官的炎症、血管功能障碍及纤维化。SSc在皮肤和器官受累程度以及疾病进展速度方面临床差异很大。最近的研究表明，内皮素系统，尤其是ET-1以及ETA和ETB受体可能在SSc的发病机制中起关键作用。一类新型药物，即内皮素受体拮抗剂已被用于治疗肺动脉高压（PAH）患者。波生坦，一种双重内皮素受体拮抗剂，以及西他生坦和安立生坦，ETA受体的选择性阻滞剂，已被证明对SSc-PAH有效。这种作用可能通过血管舒张和抗纤维化作用介导，因此使这些药物成为SSc潜在疾病修饰药物具有吸引力。
