Murdaca Giuseppe, Spanò Francesca, Contatore Miriam, Guastalla Andrea, Puppo Francesco
Department of Internal Medicine, Clinical Immunology Unit, University of Genova, Viale Benedetto XV, n. 6, 16132 Genova, Italy.
Immunotherapy. 2014;6(3):283-9. doi: 10.2217/imt.13.173.
Systemic sclerosis (SSc) is a rare connective tissue disease characterized by chronic inflammation and fibrosis of the skin, vascular abnormalities and variable involvement of organs. TNF-α has a central role in initial host response to infections and in the pathogenesis of various systemic immune-mediated diseases. Serum levels of TNF-α are elevated in patients with SSc and favor the development of pulmonary fibrosis and pulmonary arterial hypertension. Inflammatory arthritis can occur in patients with SSc. Infliximab and etanercept may improve the inflammatory arthritis and disability in SSc. TNF-α inhibitors reduce the systemic inflammation, improve the endothelial function decreasing the risk of pulmonary arterial hypertension progression and of acute cardiovascular and/or cerebrovascular events. Physicians need to be aware of the potential risks of tuberculosis reactivation and opportunistic infections. Randomized controlled trials with TNF-α inhibitors in patients with SSc are needed to confirm the potential role of these agents in the treatment of SSc.
系统性硬化症（SSc）是一种罕见的结缔组织疾病，其特征为皮肤慢性炎症和纤维化、血管异常以及器官不同程度受累。肿瘤坏死因子-α（TNF-α）在宿主对感染的初始反应以及各种系统性免疫介导疾病的发病机制中起核心作用。SSc患者血清TNF-α水平升高，这有利于肺纤维化和肺动脉高压的发展。SSc患者可发生炎性关节炎。英夫利昔单抗和依那西普可能改善SSc患者的炎性关节炎和功能障碍。TNF-α抑制剂可减轻全身炎症，改善内皮功能，降低肺动脉高压进展以及急性心血管和/或脑血管事件的风险。医生需要意识到结核复发和机会性感染的潜在风险。需要对SSc患者进行TNF-α抑制剂的随机对照试验，以证实这些药物在SSc治疗中的潜在作用。
