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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