Fibrosis mechanisms in systemic sclerosis and new potential therapies.

IF 3.6 4区 医学 Q1 MEDICINE, GENERAL & INTERNAL Postgraduate Medical Journal Pub Date : 2024-12-05 DOI:10.1093/postmj/qgae169
Raffaele Barile, Cinzia Rotondo, Valeria Rella, Antonello Trotta, Francesco Paolo Cantatore, Addolorata Corrado
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Abstract

Systemic sclerosis is a rare rheumatic disease characterized by immune cell activation, tissue fibrosis, and endothelial dysfunction. Extracellular matrix synthesis disorder causes widespread fibrosis, primarily in skin and internal organs. Various factors such as TGFβ, VEGF, Galectin-3, and signaling pathways like Wnt/β-catenin are involved in pathophysiological processes. Treatment lacks a unified approach but combines diverse modalities tailored to disease subtype and progression. Current therapeutic strategies include biologics, JAK inhibitors, and IL-6 pathway modulators. Monoclonal antibodies and hypomethylating agents demonstrate potential in fibrosis inhibition. This review focuses on emerging therapeutic evidence regarding drugs targeting collagen, cytokines, and cell surface molecules in systemic sclerosis, aiming to provide insight into potential innovative treatment strategies.

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系统性硬化症的纤维化机制和新的潜在疗法。
系统性硬化症是一种罕见的风湿病,以免疫细胞活化、组织纤维化和内皮功能障碍为特征。细胞外基质合成障碍引起广泛的纤维化,主要发生在皮肤和内脏器官。多种因子如TGFβ、VEGF、Galectin-3和信号通路如Wnt/β-catenin参与了病理生理过程。治疗缺乏统一的方法,但根据疾病亚型和进展结合多种方式。目前的治疗策略包括生物制剂、JAK抑制剂和IL-6通路调节剂。单克隆抗体和低甲基化药物显示出抑制纤维化的潜力。本文综述了针对系统性硬化症中胶原蛋白、细胞因子和细胞表面分子的药物治疗的新证据,旨在为潜在的创新治疗策略提供见解。
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来源期刊
Postgraduate Medical Journal
Postgraduate Medical Journal 医学-医学:内科
CiteScore
8.50
自引率
2.00%
发文量
131
审稿时长
2.5 months
期刊介绍: Postgraduate Medical Journal is a peer reviewed journal published on behalf of the Fellowship of Postgraduate Medicine. The journal aims to support junior doctors and their teachers and contribute to the continuing professional development of all doctors by publishing papers on a wide range of topics relevant to the practicing clinician and teacher. Papers published in PMJ include those that focus on core competencies; that describe current practice and new developments in all branches of medicine; that describe relevance and impact of translational research on clinical practice; that provide background relevant to examinations; and papers on medical education and medical education research. PMJ supports CPD by providing the opportunity for doctors to publish many types of articles including original clinical research; reviews; quality improvement reports; editorials, and correspondence on clinical matters.
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