Treatments Outcomes of Refractory Cutaneous Scleroderma with Janus Kinase Inhibitors: A Systematic Review

Scleroderma is a connective tissue disorder characterized by hardening and thickening of the skin. Initial treatment involves topical and systemic corticosteroid therapies, with immunosuppressive therapy in severe and refractory cases, although none are curative and outcomes are often poor. This review evaluates the efficacy and safety of novel Janus Kinase inhibitors (JAKi) therapy use in cutaneous scleroderma refractory to conventional therapy.硬皮病（Scleroderma）是一类以皮肤硬化增厚为特征的结缔组织疾病。其初始治疗方案涵盖外用与全身用糖皮质激素疗法，针对重症及难治性病例则需辅以免疫抑制治疗，但现有治疗手段均无法实现根治，且预后往往欠佳。本综述旨在评估新型Janus激酶抑制剂（Janus Kinase inhibitors, JAKi）疗法在常规治疗难治性皮肤型硬皮病中的疗效与安全性。