Raynaud's and Scleroderma Programme, NIHR Biomedical Research Centre, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK; St James University Hospital, Leeds Teaching Hospitals NHS Trust, Beckett Street, Leeds, LS9 7TF, UK.
Raynaud's and Scleroderma Programme, NIHR Biomedical Research Centre, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
Best Pract Res Clin Rheumatol. 2021 Sep;35(3):101686. doi: 10.1016/j.berh.2021.101686. Epub 2021 Apr 21.
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.
硬皮病是一种涉及多个器官的慢性自身免疫性疾病，胃肠道（GI）系统非常常见；然而，有关肛门括约肌受累和随之而来的粪便失禁（FI）的数据不足。Engel 及其同事于 1994 年首次报道了硬皮病中的 FI，但它对增加的医疗保健费用和生活质量（QoL）下降的影响尚未确定。多达 40%的胃肠道受累患者抱怨 FI，但由于大多数研究是回顾性的，且为病例报告或小样本研究系列，因此现有数据的质量较差。肛门直肠超声成像显示肛门内括约肌肌层直接受累，提示内括约肌变薄、萎缩或有疤痕。硬皮病失禁的治疗指南主要是对症治疗，根治性手术的结局较差。骶神经调节在一部分患者中取得了良好的效果，但需要更大规模的对照研究来评估其对症状和预后的疗效。
