Department of Dermatology, University Medical Center, Erlangen, Germany.
J Dtsch Dermatol Ges. 2019 Jul;17(7):716-728. doi: 10.1111/ddg.13887.
Systemic scleroderma/systemic sclerosis (SSc) is an inflammatory connective tissue disease clinically characterized by two major subtypes: limited and diffuse SSc. While both conditions present with Raynaud's phenomenon (paroxysmal digital ischemia), diffuse SSc is associated with rapid disease progression and early - prognostically relevant - involvement of internal organs. Treatment is challenging. In addition to general lifestyle modifications, measures include treatments aimed at improving circulation as well as immunosuppressive and immunomodulatory drugs. However, these agents are effective only in terms of slowing disease progression.
系统性硬皮病/系统性硬化症(SSc)是一种炎症性结缔组织疾病,临床上主要有两种亚型:局限性和弥漫性 SSc。虽然这两种情况都表现为雷诺现象(阵发性手指缺血),但弥漫性 SSc 与疾病快速进展以及早期与预后相关的内脏器官受累有关。治疗具有挑战性。除了一般的生活方式改变外,措施还包括旨在改善循环以及免疫抑制和免疫调节药物的治疗。然而,这些药物仅在减缓疾病进展方面有效。