Barrett's metaplasia and adenocarcinoma of the esophagus in scleroderma

Gastroesophageal reflux is well documented in scleroderma, but the complications of Barrett's metaplasia and adenocarcinoma are not well described. The records of 75 patients with scleroderma seen over a four-year period at the Hospital of the University of Pennsylvania were retrospectively reviewed to determine the prevalence of Barrett's metaplasia and adenocarcinoma of the esophagus and to identify clinical, manometric, laboratory, or radiographic criteria that might predict the presence of these lesions. Twenty-four of these patients underwent endoscopy. In this group, the prevalence of Barrett's metaplasia was 37 percent (nine patients) and adenocarcinoma was also present in two of these patients. The patients with and without Barrett's metaplasia were similar in age (range, 22 to 64 compared with 28 to 79, respectively), sex (six of nine compared with 12 of 15 female, respectively), frequency of esophageal motility disorders, presence of proximal skin involvement, digital ulceration, and pulmonary involvement as measured by diffusion capacity. Barrett's metaplasia was diagnosed on the basis of double-contrast esophagographic results in only one of eight patients with Barrett's metaplasia so-studied. Patients with Barrett's metaplasia tended to have longer duration of heartburn (90 ± 40 months compared with 11 ± 35 months) and dysphagia (39 ± 22 months compared with 7 ± 3 months). Patients with Barrett's metaplasia also tended to have greater impairment of lower esophageal sphincter pressure either at end-expiration (4.0 ± 2.1 compared with 6.1 ± 1.8 mm Hg) or mid-respiration (13.0 ± 3.0 compared with 16.9 ± 2.5 mm Hg). Using chi-square analysis, however, none of these differences reached statistical significance. Discrimination did occur on the basis of the presence of the CREST (calcinosis, Raynaud's phenomenon, esophageal manifestations of scleroderma, sclerodactyly, and telangiectasis) variant (55 percent compared with 7 percent, p < 0.01), a duration of dysphagia of more than five months (p < 0.03), and mid-respiratory lower esophageal sphincter pressure of less than 10 mm Hg (p < 0.05). It is suggested that: (1) Barrett's metaplasia of the esophagus occurs in one third of patients with scleroderma; (2) clinical, manometric, laboratory, and radiographic features are poor predictors of the presence of Barrett's metaplasia; (3) patients with CREST syndrome, prolonged dysphagia, or a very low lower esophageal sphincter pressure may have an increased risk for the development of metaplasia; (4) patients with scleroderma and Barrett's metaplasia have an increased risk of complications such as stricture or adenocarcinoma. A prospective study of patients with scleroderma and the development of Barrett's metaplasia is indicated.