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Tuesday, December 29, 2015

Scleroderma (Progressive Systemic Sclerosis)

Scleroderma (Progressive Systemic Sclerosis)

Pathogenesis. As many as 80 to 90% of patients with scleroderma will develop diminished esophageal peristalsis from the atrophy and fibrosis of the esophageal smooth muscle.
Clinical Presentation. Although there is dysphagia, the main clue to the diagnosis is simply the presence of gastroesophageal reflux symptoms in a person with a history of scleroderma. The LES will neither contract nor relax and basically assumes the role of an immobile open tube.
Diagnosis. Barium studies are generally unnecessary. The most accurate diagnostic test is motility studies.
Treatment. Therapy is with proton-pump inhibitors, such as omeprazole. Metoclopramide is a promotility agent that has some modest efficacy.
 

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