Scleroderma

Clinical

Scleroderma is a collagen-vascular disease characterized by diffuse fibrosis, degenerative changes, and vascular abnormalities in the skin, articular structures and other organs (kidneys, lung, heart, gastrointestinal and skeletal muscles). Esophageal involvement occurs in 75 to 87% of patients with scleroderma. In the esophagus, aperistalsis results from atrophy or cellular disruption of esophageal smooth muscle including the LES. These changes are primarily limited to the lower two-thirds of the esophagus because the upper third is composed of striated muscle. Patients often present with symptoms of reflux secondary to LES incompetence and dysphagia may result from abnormal motility, reflux esophagitis or peptic stricture formation.

Radiological findings

Barium studies may demonstrate a normal stripping wave that clears the upper esophagus, but stops at the level of the aortic arch. This is can be attributed to the striated muscle that composes the upper third of the esophagus. In the lower two-thirds, muscular contractions are weak and uncoordinated and eventually progress to aperistalsis. When in the recumbent position, barium remains static in the dilated, atonic esophagus. In contrast to achalasia, when the patient is seated upright the barium readily flows through the widely patent and dysfunctional LES. Related findings may include the presence of a hiatal hernia, reflux esophagitis and peptic strictures.