Department of Cellular Biology, Genetics, Histology and Pharmacology, Faculty of Health Sciences, University of Valladolid, Campus of Soria, 42004 Soria, Spain.
Neurobiology Research Group, Faculty of Medicine, University of Valladolid, 47005 Valladolid, Spain.
Medicina (Kaunas). 2023 Jan 27;59(2):247. doi: 10.3390/medicina59020247.
Scleroderma or systemic sclerosis (SSc) is an autoimmune disease affecting the connective tissue, characterized by fibrosis of the skin and internal organs. There is currently no curative treatment available, so therapeutic action is aimed at a symptomatic treatment of the affected organs. The development of biotechnology has made it possible to implement certain biological drugs that could represent a window of opportunity to modulate the evolution and symptomatology of scleroderma with greater efficacy and less toxicity than conventional treatments. This study aimed to review the current evidence critically and systematically on the effects of biological drugs on the pulmonary function, skin disease, and health status of patients afflicted by diffuse cutaneous systemic sclerosis (dcSSc). Three electronic databases (Pubmed, Dialnet, and Cochrane Library Plus) were systematically searched until the cut-off date of October 2022. The review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and included original articles in English and Spanish with a controlled trial design, comparing biological drug treatments (tocilizumab, belimumab, riociguat, abatacept, and romilkimab) with a control group. The methodological quality of the studies was assessed using the McMaster quantitative form and the PEDro scale. A total of 383 studies were identified, 6 of them met the established criteria and were included in the present systematic review. A total of 426 patients treated with tocilizumab, belimumab, riociguat, abatacept, and romilkimab were included. The results showed substantial non-significant ( < 0.05) improvement trends after treatment with the biological drugs included in this review for the modified Rodnan Scale Value, Forced Vital Capacity, and Carbon Monoxide Diffusion Test; however, no benefits were shown on the Health Assessment Questionnaire-Disability Index when compared to the control group. Biological drugs, therefore, maybe a new therapeutic strategy for dcSSc and could be recommended as an additional and/or adjunctive treatment that promotes anti-fibrotic activity. This review could further define the clinical rationale for the use of biologics in the treatment of dcSSc and could provide key details on the study protocol, design, and outcome reporting.
硬皮病或系统性硬化症（SSc）是一种影响结缔组织的自身免疫性疾病，其特征是皮肤和内部器官纤维化。目前尚无治愈方法，因此治疗作用旨在针对受影响器官进行对症治疗。生物技术的发展使得实施某些生物药物成为可能，这些药物可能代表着一个机会之窗，可以比传统治疗更有效地调节硬皮病的演变和症状，同时降低毒性。本研究旨在批判性和系统地回顾当前关于生物药物对弥漫性皮肤系统性硬化症（dcSSc）患者肺功能、皮肤病和健康状况影响的证据。系统地检索了三个电子数据库（Pubmed、Dialnet 和 Cochrane Library Plus），直到 2022 年 10 月截止日期。该综述遵循系统评价和荟萃分析的首选报告项目（PRISMA）指南，并包括比较生物药物治疗（托珠单抗、贝利尤单抗、利奥西呱、阿巴西普和罗米利单抗）与对照组的英语和西班牙语原始文章。使用麦克马斯特定量表格和 PEDro 量表评估研究的方法学质量。共确定了 383 项研究，其中 6 项符合既定标准，纳入本系统评价。共纳入了 426 名接受托珠单抗、贝利尤单抗、利奥西呱、阿巴西普和罗米利单抗治疗的患者。结果显示，在接受本综述中包含的生物药物治疗后，改良罗德纳量表值、用力肺活量和一氧化碳弥散试验有实质性但无统计学意义的（<0.05）改善趋势；然而，与对照组相比，健康评估问卷残疾指数没有显示出获益。因此，生物药物可能是 dcSSc 的一种新的治疗策略，并可被推荐作为一种额外的治疗方法，促进抗纤维化活性。本综述可以进一步确定生物制剂治疗 dcSSc 的临床原理，并提供关于研究方案、设计和结果报告的关键细节。
