Systemic sclerosis-related fecal incontinence: a scoping review focusing on a neglected manifestation

This scoping review sought to summarize the current knowledge on the epidemiology, pathogenesis and clinical presentation of, and the investigations that may help characterize faecal incontinence (FI) in patients with SSc.

The planned scoping review was based on the methodological framework proposed by Arksey and O'Malley. Two databases were screened: PubMed (Medline), (Web of Science), and data extraction was performed using a predefined template.

A total of 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 extracellular matrix deposition with fibrous replacement of the internal sphincter were the key underlaying pathogenic events. The most represented patient-reported outcome 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-diarrhoeal medications and dietary adjustments were the first step of proposed FI management in included narrative reviews and guidelines.

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.