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 oesophagus.

As 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 oesophagus is affected in 80% of scleroderma cases. Symptoms include heartburn and dysphagia. 

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

The differential diagnosis includes other causes of a dilated oesophagus (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.

Radiographic features
  • 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 (antimesenteric border, focal dilatations, pseudo-diverticula)
Differential diagnoses

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

Radiographic features
  • pseudosacculation
  • loss of haustration
  • colonic dilatation
  • reduced colonic transit time
Differential diagnoses

 

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Article information

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

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Cases and figures

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    Case 2
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    Case 2: Hidebound sign
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    Scleroderma with ...
    Case 3: complicated by oesophageal cancer
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    Case 3: complicated by peptic stricture
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    Case 5
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     Case 6: duodenal involvement of scleroderma
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