Scleroderma causes abnormalities of the autonomic nervous system, microvasculature, and immune system, and causes smooth muscle atrophy and fibrosis. Though patients with scleroderma often present with skin changes, calcinosis, and Raynaud's phenomenon, 90% have gastrointestinal involvement. Half are symptomatic. Esophageal abnormalities are the most common gastrointestinal abnormality, with manometry showing low amplitude or absent peristalsis in the middle and distal esophagus and a low LES pressure. The proximal one third of the esophagus, with its striated muscle, is spared.
Symptoms include heartburn (Acid Reflux), dysphagia due to stricture or esophageal dysmotility, and pain due to diffuse spasm.
Endoscopic findings include a patulous GEJ, esophagitis, and even Barrett's esophagus due to the hypotensive LES, stricture due to reflux, a hypotonic esophagus, or Candida esophagitis due to stasis.
Retroflexed view showing a patulous gastroesophageal junction (GEJ) in a patient with scleroderma.
Although Ling and Johnston found that endoscopy was not a sensitive means to evaluate the esophagus in connective tissue disease patients, especially when compared with manometry, at videoendoscopy, Cameron et al. noted that the LES remained widely open in 12 of 13 patients with scleroderma. Ten of 13 scleroderma patients showed a typical motility abnormality consisting of a normal contraction in the upper esophagus and no contraction in the middle and distal esophagus.
Scleroderma causes abnormalities of the autonomic nervous system, microvasculature, and immune system, and causes smooth muscle atrophy and fibrosis. Though patients with scleroderma often present with skin changes, calcinosis, and Raynaud's phenomenon, 90% have gastrointestinal involvement. Half are symptomatic. Esophageal abnormalities are the most common gastrointestinal abnormality, with manometry showing low amplitude or absent peristalsis in the middle and distal esophagus and a low LES pressure. The proximal one third of the esophagus, with its striated muscle, is spared.
Symptoms include heartburn (Acid Reflux), dysphagia due to stricture or esophageal dysmotility, and pain due to diffuse spasm.
Endoscopic findings include a patulous GEJ, esophagitis, and even Barrett's esophagus due to the hypotensive LES, stricture due to reflux, a hypotonic esophagus, or Candida esophagitis due to stasis.
Retroflexed view showing a patulous gastroesophageal junction (GEJ) in a patient with scleroderma.
Although Ling and Johnston found that endoscopy was not a sensitive means to evaluate the esophagus in connective tissue disease patients, especially when compared with manometry, at videoendoscopy, Cameron et al. noted that the LES remained widely open in 12 of 13 patients with scleroderma. Ten of 13 scleroderma patients showed a typical motility abnormality consisting of a normal contraction in the upper esophagus and no contraction in the middle and distal esophagus.