Scleroderma (gastrointestinal manifestations)

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.

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

Barium study

• 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 mesenteric border

• large bowel:

  • pseudosacculation

  • 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

  • esophageal malignancy

  • esophageal stricture

• small bowel:

  • sprue: segmentation, flocculation, hypersecretion

  • small bowel obstruction

• large bowel:

  • pseudo-obstruction

  • adult Hirschsprung disease

See also

• musculoskeletal manifestations of scleroderma

• pulmonary manifestations of scleroderma

• cardiac manifestations of scleroderma

• hepatobiliary manifestations of scleroderma

• renal manifestations of scleroderma