Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Department of Rheumatology, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, PA, USA.
Expert Rev Clin Immunol. 2024 Jun;20(6):603-622. doi: 10.1080/1744666X.2024.2320205. Epub 2024 Feb 26.
Systemic sclerosis (SSc) is a connective tissue disease with heterogeneous presentation. Gastrointestinal (GI) complications of SSc are characterized by esophageal reflux, abnormal motility, and microbiome dysbiosis, which impact patient quality of life and mortality. Preventative therapeutics are lacking, with management primarily aimed at symptomatic control.
A broad literature review was conducted through electronic databases and references from key articles. We summarize the physiology of gastric acid production and GI motility to provide context for existing therapies, detail the current understanding of SSc-GI disease, and review GI medications studied in SSc. Finally, we explore new therapeutic options. We propose a management strategy that integrates data on drug efficacy with knowledge of disease pathophysiology, aiming to optimize future therapeutic targets.
SSc-GI complications remain a challenge for patients, clinicians, and investigators alike. Management presently focuses on treating symptoms and minimizing mucosal damage. Little evidence exists to suggest immunosuppressive therapy halts progression of GI involvement or reverses damage, leaving many unanswered questions about the optimal clinical approach. Further research focused on identifying patients at risk for GI progression, and the underlying mechanism(s) that drive disease will provide opportunities to prevent long-term damage, and significantly improve patient quality of life.
系统性硬化症（SSc）是一种具有异质性表现的结缔组织疾病。SSc 的胃肠道（GI）并发症的特征为胃酸反流、运动异常和微生物组失调，这会影响患者的生活质量和死亡率。目前缺乏预防性治疗方法，主要治疗方法是针对症状进行控制。
通过电子数据库和主要文章的参考文献进行了广泛的文献综述。我们总结了胃酸产生和 GI 蠕动的生理学知识，为现有治疗方法提供了背景，详细介绍了 SSc-GI 疾病的现有认识，并综述了在 SSc 中研究的 GI 药物。最后，我们探讨了新的治疗选择。我们提出了一种管理策略，将药物疗效数据与疾病病理生理学知识相结合，旨在优化未来的治疗目标。
SSc-GI 并发症仍然是患者、临床医生和研究人员共同面临的挑战。目前的管理重点是治疗症状和尽量减少黏膜损伤。几乎没有证据表明免疫抑制疗法能阻止 GI 受累的进展或逆转损伤，这就留下了许多关于最佳临床方法的未解决问题。进一步的研究集中在识别有发生 GI 进展风险的患者，以及驱动疾病的潜在机制，这将为预防长期损伤并显著改善患者的生活质量提供机会。
