Crohn's disease of the esophagus is rare but is being detected more frequently because of the use of upper endoscopy. Clinicopathologic correlation is required to establish the correct diagnosis. We present a case of esophageal Crohn's disease and review the literature to demonstrate that esophageal involvement is usually associated with disease elsewhere in the gut.
Crohn's disease of the esophagus is rare. However, because of more frequent use of upper endoscopy, foregut Crohn's disease has been shown to be more common than previously suspected. The estimated incidence in adult patients with Crohn's disease is 0.3 to 2%. (1) Esophageal involvement is more common in the pediatric population. (2-5) Therefore, upper endoscopy, together with multiple biopsies, is important not only in the evaluation of patients with Crohn's disease, but also in establishing the diagnosis. We present the endoscopic and pathologic findings of an illustrative case of esophageal Crohn's disease and review the relevant literature.
A 42-year-old man with a history of chronic colitis presented with weight loss, dysphagia, and odynophagia. Upper endoscopy revealed continuous "cobblestoning" and linear ulcers of his esophageal mucosa (figure 1) in the distal 20-cm segment, and a normal stomach and duodenum. A mucosal biopsy revealed chronic active esophagitis (figure 2) with noncaseating granulomas and an absence of microorganisms on special stains, which supported a diagnosis of Crohn's disease. Biopsies of the gastric and duodenal mucosa were normal. Colonoscopic examination with biopsies through the colon and terminal ileum revealed changes consistent with Crohn's disease in the sigmoid colon and proximal transverse colon. This patient was treated with a proton pump inhibitor, a short course of prednisone, and a maintenance regimen that included azathioprine and mesalamine. His esophageal symptoms resolved.
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As illustrated by this case, most reported cases of Crohn's disease of the esophagus are associated with disease elsewhere in the gut. (6-8) Inflammation in the esophageal mucosa usually parallels that seen in other sites of the gastrointestinal tract. However, there are rare reports in which Crohn's disease presented primarily in the esophagus? n Patients with esophageal involvement usually complain of heartburn, odynophagia, dysphagia, and substernal or epigastric pain.
Endoscopic findings have been classified into an inflammatory stage (stage 1) followed by stenosis (stage 2) caused by chronic inflammation and/or fibrosis. Early changes include aphthous ulceration, which may extend up into the hypopharynx and oral cavity. Typically, the lower part of the esophagus is more severely affected. Although apthoid ulcers and a cobblestone pattern of the mucosa are characteristic appearances seen at endoscopy, esophageal changes more often are nonspecific (e.g., hyperemia, granularity, friability, mucosal fold thickening, and nodularity). Clinicopathologic correlation is therefore required. Endoscopic ultrasonography can help demonstrate transmural and adjacent soil tissue involvement. (12) Complications may include esophageal stenosis, obstruction, fistula formation, perforation and, rarely, the development of adenocarcinoma. (13-15) Severe disease may also mimic carcinoma. Thus, biopsies should be taken to exclude not only neoplasia, but also severe reflux esophagitis and infections.
Therapy is based largely on the administration of corticosteroids. (16-17) Concomitant use of a proton pump inhibitor or sucralfate may also provide partial relief of symptoms. Opportunistic infections involving the esophagus should always remain a consideration in immunosuppressed patients with Crohn's disease. More complicated and refractory disease may warrant endoscopic dilatation or surgical resection. For patients with steroid-resistant or steroid-dependent disease, the use of infliximab (a monoclonal antibody to TNF-[alpha] may help induce and sustain clinical remission, thus avoiding surgery. (18)
Involvement of the upper gastrointestinal tract by Crohn's disease is usually underdiagnosed. (19-21) Patients with a diagnosis of Crolm's disease and esophageal symptoms should undergo upper endoscopy with biopsies to establish the cause of their symptoms, If diagnostic findings such as granulomas are not to be missed, biopsies should be taken from both ulcers and normal-appearing mucosa. Treatment with acid suppression medications and a short course of steroids is warranted. Occasionally, immunomodulators or infliximab may help sustain clinical remission.
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From the Department of Pathology (Dr. Pantanowitz and Dr. Nasser) and the Department of Gastroenterology (Dr. Gelrud and Dr. Apstein), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston.
Reprint requests: Liron Pantanowitz, MD, Department of Pathology, Beth Israel Deaconess Medical Center, 330 Brookline Ave., Boston, MA 02215. Phone: (617) 667-4344; fax: (617) 667 7120; e-mail: firstname.lastname@example.org
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