Scleroderma (gastrointestinal manifestations)
Updates to Article Attributes
Gastrointestinal manifestations of scleroderma can occur in up to 90% of patients with scleroderma 2 with the commonest site of GIgastrointestinal involvement being the oesophagus.
As the clinical presentation, radiographic appearances and differential diagnosis vary with the location of involvement these are discussed sequentially by region (see below).
For a general discussion of scleroderma, please refer to the parent article: scleroderma.
Pathology
Smooth muscle atrophy and fibrosis are thought to be the chief underlying mechanism which leads to luminal dilatation, reduced motility and reduced sphincter tone.
Oesophagus
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 the lower oesophagus (normal peristalsis above aortic arch)
- gastro-oesophageal reflux due to reduced sphincter tone
- air-fluid level in the oesophagus when supine (CT)
Complications
- 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 diagnoses
The differential diagnosis includes other causes of a dilated oesophagus (see achalasia pattern) and includes:
-
achalasia: distal segment
ofnarrowing is less than 3.5 cm - central and peripheral neuropathy
- oesophageal malignancy
- oesophageal stricture
Stomach
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.
Small bowel
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 (often on the mesenteric border)
Differential diagnoses
- sprue: segmentation, flocculation, hypersecretion
- small bowel obstruction
Large bowel
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 diagnosis
See also
-<p><strong>Gastrointestinal manifestations of scleroderma </strong>can occur in up to 90% of patients with <a href="/articles/scleroderma">scleroderma</a> <sup>2 </sup>with the commonest site of GI involvement being the <a href="/articles/oesophagus">oesophagus</a>.</p><p>As the clinical presentation, radiographic appearances and differential diagnosis vary with the location of involvement these are discussed sequentially by region (see below)</p><h4>Pathology</h4><p>Smooth muscle atrophy and fibrosis are thought to be the chief underlying mechanism which leads to luminal dilatation, reduced motility and reduced sphincter tone.</p><h4>Oesophagus</h4><p>The oesophagus is affected in 80% of scleroderma cases. Symptoms include heartburn and dysphagia. </p><h5>Radiographic features</h5><ul>- +<p><strong>Gastrointestinal manifestations of scleroderma </strong>can occur in up to 90% of patients with <a href="/articles/scleroderma">scleroderma</a> <sup>2 </sup>with the commonest site of gastrointestinal involvement being the <a href="/articles/oesophagus">oesophagus</a>.</p><p>As the clinical presentation, radiographic appearances and differential diagnosis vary with the location of involvement these are discussed sequentially by region.</p><p>For a general discussion of scleroderma, please refer to the parent article: <a href="/articles/scleroderma">scleroderma</a>.</p><h4>Pathology</h4><p>Smooth muscle atrophy and fibrosis are thought to be the chief underlying mechanism which leads to luminal dilatation, reduced motility and reduced sphincter tone.</p><h4>Oesophagus</h4><p>The oesophagus is affected in 80% of scleroderma cases. Symptoms include heartburn and dysphagia. </p><h5>Radiographic features</h5><ul>
-<li>dysmotility of lower oesophagus (normal peristalsis above aortic arch)</li>- +<li>dysmotility of the lower oesophagus (normal peristalsis above aortic arch)</li>
-<li>air-fluid level in oesophagus when supine (CT)</li>- +<li>air-fluid level in the oesophagus when supine (CT)</li>
-<a href="/articles/achalasia">achalasia</a>: distal segment of narrowing is less than 3.5 cm</li>- +<a href="/articles/achalasia">achalasia</a>: distal segment narrowing is less than 3.5 cm</li>
-<li><a href="/articles/scleroderma-pulmonary-manifestations-1">pulmonary manifestations of scleroderma</a></li>- +<li><a href="/articles/scleroderma-pulmonary-manifestations-1">pulmonary manifestations of scleroderma</a></li>
- +<li><a href="/articles/cardiac-manifestations-of-scleroderma">cardiac manifestations of scleroderma</a></li>
- +<li><a href="/articles/scleroderma-hepatobiliary-manifestations-1">hepatobiliary manifestations of scleroderma</a></li>
- +<li><a href="/articles/scleroderma-renal-manifestations-1">renal manifestations of scleroderma</a></li>
Systems changed:
- Oncology
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