Systemic sclerosis is a systemic fibrosing disorder associated with significant morbidity and mortality, with no universally accepted disease-modifying therapy. Significant advances in the understanding of systemic sclerosis in recent years have guided the exploration of biological drugs in systemic sclerosis. In this narrative review, we summarize the published literature on biologic therapies in systemic sclerosis. A double-blind randomized trial, and an open label trial of tocilizumab (which antagonizes the interleukin 6 receptor), identified potential benefits in skin and lung fibrosis in systemic sclerosis; however, these differences failed to attain statistical significance. Two open-label trials compared rituximab (which depletes B lymphocytes) to conventional treatment/ cyclophosphamide in systemic sclerosis-associated interstitial lung disease (ILD), and revealed significant improvements in lung functions and skin disease with rituximab. Significant observational data also support the use of rituximab in skin, lung, muscle and joint manifestations of systemic sclerosis. Abatacept (which blocks T lymphocyte activation) has demonstrated utility for skin and joint disease in systemic sclerosis; a recent clinical trial failed to demonstrate benefits in improving skin thickness compared to placebo. Agents targeting type I interferons, interleukin 17 pathway, CD19 and plasma cells hold promise in systemic sclerosis; however, high-quality evidence is lacking. The results of different ongoing clinical trials targeting B lymphocytes, T lymphocytes, various cytokines (interleukins 6, 17, 4, 13, IL-1α), platelet-derived growth factor receptor, proteasome, integrins or oncostatin M may help guide future therapeutic regimens with biological agents in systemic sclerosis.

中文翻译：

---

全身性硬化症中的生物疗法是答案吗？

系统性硬化症是一种系统性纤维化疾病，伴有明显的发病率和死亡率，尚无普遍接受的改善疾病的疗法。近年来，在对系统性硬化症的理解上的重大进展指导了对系统性硬化症的生物药物的探索。在这篇叙述性综述中，我们总结了有关系统性硬化症生物疗法的已发表文献。一项双盲随机试验和托珠单抗的开放标签试验（拮抗白介素6受体）确定了系统性硬化症对皮肤和肺纤维化的潜在益处；但是，这些差异未能达到统计学意义。两项开放性试验比较了利妥昔单抗（消耗B淋巴细胞）与常规治疗/环磷酰胺治疗系统性硬化症相关性间质性肺病（ILD）的情况，并显示利妥昔单抗显着改善了肺功能和皮肤疾病。重要的观察数据也支持利妥昔单抗在全身性硬化症的皮肤，肺，肌肉和关节表现中的使用。已证明Abatacept（可阻断T淋巴细胞活化）可用于全身性硬化症中的皮肤和关节疾病。一项最新的临床试验未能证明与安慰剂相比，在改善皮肤厚度方面具有益处。靶向I型干扰素，白介素17途径，CD19和浆细胞的药物有望在全身性硬化症中发挥作用。但是，缺乏高质量的证据。