Scleroderma (gastrointestinal manifestations)

Gastrointestinal manifestations of scleroderma can occur in up to 90% of patients with scleroderma 2 with the commonest site of GI involvement being the esophagus.

As the clinical presentation, radiographic appearances and differential diagnosis vary with the location of involvement these are discussed sequentially by region (see below)

Smooth muscle atrophy and fibrosis are thought to be the chief underlying mechanism which leads to luminal dilatation, reduced motility and reduced sphincter tone.

The esophagus is affected in 80% of scleroderma cases. Symptoms include heartburn and dysphagia. 

  • dilatation of distal two-thirds of the esophagus 1
  • apparent shortening of length due to fibrosis
  • dysmotility of lower esophagus (normal peristalsis above aortic arch)
  • gastro-esophageal reflux due to reduced sphincter tone
  • air-fluid level in esophagus when supine (CT)

The differential diagnosis includes other causes of a dilated esophagus (see achalasia pattern) and includes:

Gastric involvement is relatively uncommon but can result in delayed gastric emptying with or without gastric dilatation. Gastric vascular antral ectasia (dilated submucosal capillaries), often known as watermelon stomach, may also occur.

The small bowel is affected in more than 60% of scleroderma patients, the duodenum most frequently. Patients may be asymptomatic or may present with bloating or malabsorption due to bacterial overgrowth.

  • luminal dilatation (can be massive)
  • reduced peristalsis / delayed contrast transit
  • mucosal folds appear relatively normal despite dilatation
  • hidebound bowel sign (crowding of valvulae conniventes): thought to be pathognomonic of scleroderma
  • accordion sign: well seen evenly spaced mucosal folds in duodenum
  • sacculation (often on the mesenteric border)

The large bowel is affected in ~40% of patients and may cause constipation or diarrhea. Reduced anal sphincter tone can result in fecal incontinence. 

  • pseudosacculation
  • loss of haustration
  • colonic dilatation
  • reduced colonic transit time
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Article information

rID: 8607
Synonyms or Alternate Spellings:
  • Gastro-intestinal manifestations of scleroderma

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Cases and figures

  • Case 1
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  • Case 2
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  • Case 2: Hidebound sign
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  • Case 3: complicated by esophageal cancer
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  • Case 3: complicated by peptic stricture
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  • Case 4
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  • Case 5: duodenal involvement of scleroderma
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  • Case 6: esophageal dysmotility
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