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硬皮病的治疗。

Treatment of scleroderma.

作者信息

Sapadin Allen N, Fleischmajer Raul

机构信息

Department of Dermatology, Mount Sinai School of Medicine, 1425 Madison Ave, PO Box 1047, New York, NY 10029, USA.

出版信息

Arch Dermatol. 2002 Jan;138(1):99-105. doi: 10.1001/archderm.138.1.99.

Abstract

The treatment of systemic sclerosis (scleroderma) is difficult and remains a great challenge to the clinician. Because the cause is unknown, therapies are directed to improve peripheral blood circulation with vasodilators and antiplatelet aggregation drugs, to prevent the synthesis and release of harmful cytokines with immunosuppressant drugs, and to inhibit or reduce fibrosis with agents that reduce collagen synthesis or enhance collagenase production. The purpose of this review is to critically analyze conventional and new treatments of systemic sclerosis and localized scleroderma. The therapeutic options discussed for the treatment of systemic sclerosis include the use of (1) vasodilators (calcium channel blockers [nifedipine], angiotensin-converting enzyme inhibitors [captopril, losartan potassium], and prostaglandins [iloprost, epoprostenol]), (2) immunosuppressant drugs (methotrexate, cyclosporine, cyclophosphamide, and extracorporeal photopheresis), and (3) antifibrotic agents (D-penicillamine, colchicine, interferon gamma, and relaxin). The treatment options reviewed for localized scleroderma include the use of corticosteroids, vitamin D analogues (calcitriol, calcipotriene), UV-A, and methotrexate. Preliminary reports on new therapies for systemic sclerosis are also considered. These include the use of minocycline, psoralen-UV-A, lung transplantation, autologous stem cell transplantation, etanercept, and thalidomide.

摘要

系统性硬化症(硬皮病)的治疗颇具难度,对临床医生而言仍是巨大挑战。由于病因不明,治疗方法旨在通过血管扩张剂和抗血小板聚集药物改善外周血液循环,通过免疫抑制剂药物防止有害细胞因子的合成与释放,并通过减少胶原蛋白合成或增强胶原酶生成的药物抑制或减轻纤维化。本综述的目的是批判性地分析系统性硬化症和局限性硬皮病的传统及新治疗方法。讨论的系统性硬化症治疗选择包括使用(1)血管扩张剂(钙通道阻滞剂[硝苯地平]、血管紧张素转换酶抑制剂[卡托普利、氯沙坦钾]和前列腺素[伊洛前列素、依前列醇]),(2)免疫抑制剂药物(甲氨蝶呤、环孢素、环磷酰胺和体外光化学疗法),以及(3)抗纤维化药物(D-青霉胺、秋水仙碱、干扰素γ和松弛素)。综述的局限性硬皮病治疗选择包括使用皮质类固醇、维生素D类似物(骨化三醇、卡泊三醇)、紫外线A和甲氨蝶呤。还考虑了关于系统性硬化症新疗法的初步报告。这些包括使用米诺环素、补骨脂素-紫外线A、肺移植、自体干细胞移植、依那西普和沙利度胺。

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