A 35-year-old woman comes to the clinic due to severe heartburn that is resistant to over-the-counter antacids. The patient has no known medical problems and takes no other medications. She occasionally has a glass of wine with dinner but does not use tobacco or illicit drugs. Physical examination shows scattered telangiectasias on the face, several ulcers at the tips of the fingers, and small calcium deposits in the soft tissues of the hands and elbows. Which of the following processes is the most likely cause of this patient's heartburn?

Question

A 35-year-old woman comes to the clinic due to severe heartburn that is resistant to over-the-counter antacids.  The patient has no known medical problems and takes no other medications.  She occasionally has a glass of wine with dinner but does not use tobacco or illicit drugs.  Physical examination shows scattered telangiectasias on the face, several ulcers at the tips of the fingers, and small calcium deposits in the soft tissues of the hands and elbows.  Which of the following processes is the most likely cause of this patient's heartburn?

Quizplus · Accepted Answer

This patient has features consistent with CREST syndrome (ie, Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia), a limited variant of systemic sclerosis with skin disease that primarily affects the face, forearms, and fingers. The pathogenesis of systemic sclerosis involves chronic autoimmune inflammation, vascular endothelial injury resulting in chronic ischemic tissue damage (eg, fingertip ulcers), and excessive activation of fibroblasts leading to progressive tissue fibrosis. Esophageal dysmotility is a result of atrophy and fibrous replacement of the muscularis in the lower esophagus. The esophageal body and the lower esophageal sphincter become atonic and dilated, resulting in symptoms of gastroesophageal reflux (eg, heartburn, regurgitation, dysphagia). This increases the risk of Barrett's esophagus and fibrous stricture formation. (Choice A) A sliding hiatal hernia is characterized by herniation of the gastroesophageal junction and a portion of the stomach through the esophageal hiatus, which predisposes patients to gastroesophageal reflux. (Choice C) Increased gastric acid production occurs with gastrinomas, gastrin-secreting neuroendocrine tumors of the small intestine/pancreas that often present with multiple or medically refractory peptic ulcers. (Choice D) Achalasia is an esophageal motility disorder characterized by failed relaxation of the lower esophageal sphincter resulting in food retention, dilation of the esophageal body, and symptoms of solid/liquid dysphagia. (Choice E) Diffuse esophageal spasm is characterized by periodic, uncoordinated, simultaneous contractions of the lower esophagus due to impaired inhibitory innervation within the esophageal myenteric plexus. Patients typically have liquid/solid dysphagia and chest pain due to inefficient propagation of food into the stomach. Educational objective: Systemic sclerosis may result in esophageal dysmotility and incompetence of the lower esophageal sphincter due to atrophy and fibrous replacement of the esophageal muscularis. This can cause gastroesophageal reflux with an increased risk of Barrett's esophagus and stricture formation. __________ References: Diagnosis and classification of systemic sclerosis. (http://www.ncbi.nlm.nih.gov/pubmed/20143182) Diagnosis and treatment of scleroderma. (http://www.ncbi.nlm.nih.gov/pubmed/18560030)

A 35-Year-Old Woman Comes to the Clinic Due to Severe