Scleroderma (gastrointestinal manifestations)

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Gastrointestinal manifestations of scleroderma can occur in up to 90% of patients with scleroderma ²with the most common site of gastrointestinal involvement being the oesophagus. 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:

oesophagus:
- 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 diarrhoea
- faecal 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:

oesophagus: 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 studyFluoroscopy

oesophagus:
- dilatation of distal two-thirds of the oesophagus ¹
- deficient oesophageal emptying in a recumbent position
- apparent shortening of length due to fibrosis
- dysmotility of the lower oesophagus (normal peristalsis above the aortic arch)
- gastro-oesophageal 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 border⁴
large bowel:
- pseudosacculation
- loss of haustration
- colonic dilatation
- reduced colonic transit time

CT

oesophagus: air-fluid level when supine
small bowel: pneumatosis cystoides intestinalis

Treatment and prognosis

Complications

Complications of oesophageal involvement include:

aspiration
oesophagitis
- mucosal erosion
- fusiform stricture ~4-5 cm above the gastro-oesophageal junction
- progression to Barrett oesophagus (~40%) ³
- higher risk of development of oesophageal cancer (adenocarcinoma)

Differential diagnosis

oesophagus: the differential diagnosis includes other causes of a dilated oesophagus (see achalasia pattern)
- achalasia: distal segment narrowing is less than 3.5 cm
- central and peripheral neuropathy
- oesophageal malignancy
- oesophageal stricture
small bowel:
- sprue: segmentation, flocculation, hypersecretion
- small bowel obstruction
large bowel:
- pseudo-obstruction
- adult Hirschsprung disease

~~-</ul><h4>Radiographic features</h4><h5>Barium study</h5><ul>~~
+</ul><h4>Radiographic features</h4><h5>Fluoroscopy</h5><ul>
~~-<p>oesophagus:</p>~~
+<p>oesophagus</p>
~~-<p>small bowel:</p>~~
+<p>small bowel</p>
~~-<li><p>sacculation: often on the anti-mesenteric border</p></li>~~
+<li><p>sacculation: often on the anti-mesenteric border <sup>4</sup></p></li>
~~-<p>large bowel:</p>~~
+<p>large bowel</p>
~~-<p>small bowel:</p>~~
+<p>small bowel</p>
~~-<p>large bowel:</p>~~
+<p>large bowel</p>

References changed:

3. Katzka D, Reynolds J, Saul S et al. Barrett's Metaplasia and Adenocarcinoma of the Esophagus in Scleroderma. Am J Med. 1987;82(1):46-52. <a href="https://doi.org/10.1016/0002-9343(87)90376-7">doi:10.1016/0002-9343(87)90376-7</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/3799692">Pubmed</a>
4. Chatzinikolaou S, Quirk B, Murray C, Planche K. Radiological Findings in Gastrointestinal Scleroderma. Journal of Scleroderma and Related Disorders. 2019;5(1):21-32. <a href="https://doi.org/10.1177/2397198319848550">doi:10.1177/2397198319848550</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/35382407">Pubmed</a>
3. Katzka DA, Reynolds JC, Saul SH et-al. Barrett's metaplasia and adenocarcinoma of the esophagus in scleroderma. Am. J. Med. 1987;82 (1): 46-52. <a href="http://www.ncbi.nlm.nih.gov/pubmed/3799692">Pubmed citation</a><span class="auto"></span>

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