Scleroderma of the Gastrointestinal Tract

General Considerations

• Gastrointestinal manifestations of scleroderma are relatively common (40-45%), following skin changes and Raynaud’s phenomena
• In the GI tract, there may be atrophy of the smooth muscle
• CD4 T cells are believed to play an important role in its pathogenesis
• The esophagus is affected most frequently (85%) with lesser changes in the stomach (uncommon), small (45%) and large bowel

Clinical Findings

• Many patients are asymptomatic despite significant GI changes
• Dysphagia
• Reflux
• Delayed gastric emptying
• Malabsorption
• Pseudo-obstruction
• Constipation

Imaging Findings

• Contrast-enhanced conventional radiography (i.e. barium studies) are usually used to image GI scleroderma
• Esophagus
• Dilated, hypomotile esophagus specially involving distal 2/3
• Patulous esophagogastric junction
• Delayed emptying of esophagus in recumbent position but improved emptying when upright (DDx: achalasia)
• Distal esophageal stricture from reflux esophagitis
• Barrett esophagus
• Adenocarcinoma from Barrett esophagus
• Stomach
• Involvement is rare but there may be dilatation and hypomotility
• Small bowel
• Dilation, especially duodenum and jejunum
• Fragmentation and flocculation of barium
• “Hide-bound” appearance with valvulae close together despite small bowel dilatation
• Pseudo-diverticula on the mesenteric side
• Colon
• Large-mouth pseudo-diverticula on anti-mesenteric border, usually in transverse and descending colons
• Dilatation
• Effacement of the haustra

Differential Diagnosis

• Achalasia
• Small bowel obstruction

 Gastrointestinal Scleroderma. A single view of the lower chest and abdomen after an upper gastrointestinal barium study demonstrates a dilated, distal esophagus with reflux (red arrow, a normal-appearing stomach and dilation of the small bowel (white arrow) with relatively close approximation of the valvulae-the "hide-bound appearance" (yellow arrow).

Gastrointestinal Scleroderma Imaging. AN Khan and R Rahim. eMedicine