Fecal incontinence and scleroderma: Pathogenesis and unmet needs.

Scleroderma is a chronic autoimmune disorder involving multiple organs and very commonly the gastrointestinal (GI) system; nevertheless, data on the involvement of the anal sphincter and consequent faecal incontinence (FI) are inadequate. FI in scleroderma was first reported in 1994 by Engel and colleagues, but its impact of added health care costs and declining quality of life (QoL) is poorly determined. Up to 40% of patients with GI involvement complain of FI, however, the quality of data available is poor owing to majority of the studies being retrospective and case reports or series of small study size. A direct involvement of internal anal sphincter muscularis propria has been demonstrated on anorectal ultrasound imaging suggesting a thin, atrophic or scarred internal sphincter. Treatment guidelines for incontinence in scleroderma are mainly symptomatic, with radical surgeries burdened by poor outcomes. Sacral neuromodulation is being used with good outcomes in a subgroup of patients, but larger, controlled studies are required to assess its efficacy on symptoms and prognosis.