Gastrointestinal manifestations of scleroderma can occur in up to 90% of patients with scleroderma 2 with the most common site of gastrointestinal involvement being the esophagus. After skin changes and Raynaud phenomenon, gastrointestinal changes are the third most common manifestation of scleroderma.
For a general discussion of scleroderma, please refer to the parent article: scleroderma.
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Clinical presentation
The clinical presentation varies with the location of involvement:
-
esophagus:
heartburn and dysphagia
-
stomach:
delayed gastric emptying with or without gastric dilatation
gastric vascular antral ectasia (dilated submucosal capillaries), often known as watermelon stomach
-
small bowel:
asymptomatic
bloating or malabsorption due to bacterial overgrowth
-
large bowel including anorectum:
constipation or diarrhea
fecal incontinence due to reduced anal sphincter tone
Pathology
Smooth muscle atrophy and fibrosis are thought to be the chief underlying mechanism that leads to luminal dilatation, reduced motility and reduced sphincter tone.
Location
Sites of involvement are:
esophagus: affected in 80% of cases
small bowel: affected in 60% of cases (most commonly at the duodenum)
large bowel: affected in 40% of cases
stomach: rare
Radiographic features
Fluoroscopy
-
esophagus
dilatation of distal two-thirds of the esophagus 1
deficient esophageal emptying in a recumbent position
apparent shortening of length due to fibrosis
dysmotility of the lower esophagus (normal peristalsis above the aortic arch)
gastro-esophageal reflux due to reduced sphincter tone
-
small bowel
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 the duodenum
sacculation: often on the anti-mesenteric border 4
-
large bowel
loss of haustration
colonic dilatation
reduced colonic transit time
CT
esophagus: air-fluid level when supine
small bowel: pneumatosis cystoides intestinalis
Treatment and prognosis
Complications
Complications of esophageal involvement include:
aspiration
-
esophagitis
mucosal erosion
fusiform stricture ~4-5 cm above the gastro-esophageal junction
progression to Barrett esophagus (~40%) 3
higher risk of development of esophageal cancer (adenocarcinoma)
Differential diagnosis
-
esophagus: the differential diagnosis includes other causes of a dilated esophagus (see achalasia pattern)
achalasia: distal segment narrowing is less than 3.5 cm
central and peripheral neuropathy
-
small bowel
sprue: segmentation, flocculation, hypersecretion
-
large bowel
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