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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


 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