: Objectives This scoping-review sought to summarize the current knowledge on the epidemiology, pathogenesis, clinical presentation, and the investigations that may help characterize faecal incontinence (FI) in patients with systemic sclerosis (SSc). Methods The planned scoping review was based on the methodological framework proposed by Arksey & O’Malley.Two databases were screened: PubMed (Medline), (webofSciences), data extraction was performed using a predefined template. Results 454 abstracts were screened and 61 articles were finally included, comprising 32 original articles. The prevalence of FI was 0,4% to 77% in original articles that did not use FI among the mandatory inclusion criteria. Internal anal sphincter was reported as more impacted than external sphincter and vasculopathy of arterioles and extra-cellular matrix deposition with fibrous replacement of the internal sphincter were the key underlaying pathogenic events. The most represented PRO in original articles was the Wexner FI score (22% of original articles) followed by the UCLA SCTC-GIT 2.0 (16% of original articles). Although there is no validated diagnostic approach for FI in SSc, 47% of original articles used anorectal manometry to assess rectal physiology in SSc patients. Conservative measures to treat either liquid or hard stool including anti-diarrheal medications and dietary adjustments were the first step of proposed FI management in included narrative reviews and guidelines. Conclusion This is the first scoping review exploring FI in SSc. We propose a new research agenda which may help improve treatment strategies and foster research focusing on a neglected manifestation of SSc.

中文翻译：

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系统性硬化症相关的大便失禁：一项针对被忽视表现的范围综述


： 目的 本范围综述旨在总结目前关于流行病学、发病机制、临床表现和可能有助于描述系统性硬化症 （SSc） 患者大便失禁 （FI） 的调查的知识。方法 计划的范围审查基于Arksey和O'Malley提出的方法框架。筛选了两个数据库： PubMed （Medline）、 （webofSciences），使用预定义的模板进行数据提取。结果 筛选出 454 篇摘要，最终纳入 61 篇文献，包括 32 篇原始文献。在强制性纳入标准中未使用 FI 的原始文章中，FI 的患病率为 0.4% 至 77%。据报道，肛门内括约肌比外括约肌受影响更大，小动脉血管病变和细胞外基质沉积伴内括约肌纤维置换是关键的潜在致病事件。在原创文章中，代表性最强的 PRO 是 Wexner FI 分数（占原始文章的 22%），其次是 UCLA SCTC-GIT 2.0（占原始文章的 16%）。尽管没有经过验证的 SSc 中 FI 的诊断方法，但 47% 的原始文章使用肛门直肠测压来评估 SSc 患者的直肠生理学。治疗液体或硬便的保守措施，包括止泻药和饮食调整，是纳入叙述性综述和指南中提议的 FI 管理的第一步。结论这是首次探讨 SSc 中 FI 的范围综述。我们提出了一个新的研究议程，这可能有助于改进治疗策略并促进针对 SSc 被忽视表现的研究。