Bohdziewicz Anna, Pawlik Katarzyna Karina, Maciejewska Magdalena, Sikora Mariusz, Alda-Malicka Rosanna, Czuwara Joanna, Rudnicka Lidia
Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, 02-008 Warsaw, Poland.
National Institute of Geriatrics, Rheumatology and Rehabilitation, Spartańska 1, 02-637 Warsaw, Poland.
J Clin Med. 2022 Feb 27;11(5):1310. doi: 10.3390/jcm11051310.
Systemic sclerosis is an autoimmune connective tissue disease characterized by vasculopathy and fibrosis of the skin and internal organs. The pathogenesis of systemic sclerosis is very complex. Mediators produced by immune cells are involved in the inflammatory processes occurring in the tissues. The currently available therapeutic options are often insufficient to halt disease progress. This article presents an overview of potential therapeutic targets and the pipeline of possible future therapeutic options. It is based on research of clinical trials involving novel, unestablished methods of treatment. Increasing knowledge of the processes and mediators involved in systemic scleroderma has led to the initiation of drug trials with therapeutic targets of CD28-CD80/86, CD19, CCL24, CD20, CD30, tumor necrosis factor (TNF), transforming growth factor β (TGF-β), B-cell activating factor (BAFF), lysophosphatidic acid receptor 1 (LPA1 receptor), soluble guanylate cyclase (sGC), Janus kinases (JAK), interleukin 6 (IL-6), endothelin receptor, and autotaxin. Data from clinical trials on these drugs indicate a significant potential for several new therapeutic options for systemic sclerosis in the upcoming future.
系统性硬化症是一种自身免疫性结缔组织疾病，其特征为血管病变以及皮肤和内脏器官的纤维化。系统性硬化症的发病机制非常复杂。免疫细胞产生的介质参与了组织中发生的炎症过程。目前可用的治疗方法往往不足以阻止疾病进展。本文概述了潜在的治疗靶点以及未来可能的治疗方案。它基于对涉及新型、未确立治疗方法的临床试验的研究。对系统性硬化症相关过程和介质的了解不断增加，已引发了针对CD28 - CD80/86、CD19、CCL24、CD20、CD30、肿瘤坏死因子（TNF）、转化生长因子β（TGF-β）、B细胞活化因子（BAFF）、溶血磷脂酸受体1（LPA1受体）、可溶性鸟苷酸环化酶（sGC）、Janus激酶（JAK）、白细胞介素6（IL-6）、内皮素受体和自分泌运动因子的药物试验。这些药物的临床试验数据表明，在不久的将来，几种新的系统性硬化症治疗方案具有巨大潜力。
