Systemic sclerosis involves the gastrointestinal tract in almost everyone, and the oesophagus in about 90%. This review's organising point is that involvement is not a single complication to be watched for but a set of them, one per segment, all flowing from the same three mechanisms — vasculopathy, immune-mediated inflammation and neuropathy — acting on smooth muscle that is atrophying and being replaced by fibrosis.
The map runs the length of the tract. Oesophagus: dysphagia and reflux. Stomach: gastroparesis and gastric antral vascular ectasia, which presents as iron deficiency and is endoscopically distinctive. Small intestine: telangiectasia, pseudo-obstruction and small intestinal bacterial overgrowth. Colon: constipation, dilatation, and in severe cases megacolon and pneumatosis cystoides intestinalis, which is alarming on imaging and usually not a surgical emergency in this context. Anorectum: faecal incontinence from thinning of the sphincters, which patients very rarely volunteer.
Two cautions govern practice. Symptoms correlate poorly with the underlying pathology and functional impairment, so a patient reporting little may have a great deal wrong, and associations with disease features and autoantibody titres are variable enough not to be relied on for prediction. And high-quality scleroderma-specific treatment evidence barely exists — nearly every management recommendation is imported from patients without scleroderma, which is worth saying to the patient rather than implying a certainty that is not there.
- Ask about faecal incontinence directly; it is common, disabling and almost never volunteered
- Investigate iron deficiency for gastric antral vascular ectasia rather than attributing it to reflux
- Have a low threshold for small intestinal bacterial overgrowth in bloating and diarrhoea
- Do not use symptom severity to gauge the extent of gut involvement
- Say explicitly that most treatment evidence is extrapolated from non-scleroderma patients
Why it matters
It breaks the link between how much a scleroderma patient complains and how much of their gut is affected.
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