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Decreased lower esophageal tone
18%
7/39
Esophageal fibrosis
64%
25/39
Increased lower esophageal tone
3%
1/39
Spastic cricopharyngeal muscle
5%
2/39
Uncoordinated esophageal peristalsis
8%
3/39
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This patient has clinical features suggestive of scleroderma given her taut skin (young-appearing skin), hand pain in the cold (Raynaud syndrome), esophageal reflux (GERD from fibrosis of the esophagus), kidney disease (suggested by her hypertension), and interstitial lung disease (as seen on CT with the diffuse fibrosis). Scleroderma is a systemic collagen vascular disease with inappropriate accumulation of collagen in various organ systems. Organ systems that can be affected include renal (presenting with hypertension, hyperkalemia, and an elevated BUN/creatinine), pulmonary (presenting with pulmonary fibrosis and dyspnea), dermatologic (presenting with taut and smooth skin as well as Raynaud disease), and GI symptoms (in particular, GERD secondary to collagen deposition in the esophagus). There is no definitive treatment of scleroderma and management involves treating each of the underlying organ systems affected. The treatment of GERD in scleroderma typically involves administration of PPIs and small meals that are low in fat. Figure A shows diffuse fibrosis of the lungs concerning for interstitial lung disease which is commonly seen in scleroderma. Incorrect Answers: Answer 1: Decreased lower esophageal tone is the pathophysiology of GERD which presents with burning retrosternal pain typically after a meal. Though it could be the cause of this patient's symptoms, her other findings suggestive of scleroderma make fibrosis a more likely etiology. Answer 3: Increased lower esophageal tone is the pathophysiology of achalasia which presents in young men with regurgitation of undigested food particles. Barium swallow would show a bird beak appearance and manometry would demonstrate increased lower esophageal tone. Treatment involves a myotomy. Answer 4: Spastic cricopharyngeal muscle describes a Zenker diverticulum which presents with regurgitation of undigested food in an elderly patient and a sensation that food is stuck in the throat. Barium swallow will show an outpouching in the proximal esophagus and treatment involves surgical resection. Answer 5: Uncoordinated esophageal peristalsis describes diffuse esophageal spasm which presents with episodic chest pain and dysphagia secondary to intermittent esophageal spasm. Treatment involves administration of calcium channel blockers. Bullet Summary: GERD is a common complication of scleroderma and occurs secondary to collagen deposition in the esophagus.
4.5
(15)
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