Lymphocytic Esophagitis Presenting as Severe Dysphagia

Abstract

Purpose: A 35-year-old African-American male with a history of childhood asthma, eczema, and food, drug and seasonal allergies presented with dysphagia. His symptoms began at age 7 years when he presented with regurgitation and dysphagia. There was no toxic ingestion. He was treated with antacids and H2 blockers. Symptoms were troublesome for years but resolved by age 10 years. He presented again at age 27 years with inability to tolerate solid foods. Upper endoscopy (EGD) at his local hospital was reportedly normal. He underwent manometry and was reportedly diagnosed with achalasia. A surgical myotomy was performed with only minimal improvement in symptoms. The patient presented to our institution with progressive severe solid dysphagia and weight loss. He denied odynophagia, fever, or night sweats. Physical exam: thin male, no oral lesions or lymphadenopathy. Labs: normal hemoglobin, electrolytes, and serum albumin. HIV negative. Barium esophagram: dilated esophagus with severe esophageal dysmotility. Repeat EGD: severely fibrotic and nodular mucosa. Biopsies caused linear tears and bleeding, requiring endoscopic clipping. Pathology showed active esophagitis and an inflammatory infiltrate composed of lymphocytes with admixed neutrophils and eosinophils. Based on the patient's history and pathologic findings, a clinical diagnosis of severe longstanding lymphocytic esophagitis (LE) or eosinophilic esophagitis (EoE) was made. A trial of high-dose PPI and topical budesonide was unsuccessful. Given the severity of the endoscopic findings and lack of response to medical therapy, the patient underwent an esophagectomy. Post-operatively he is able to tolerate a solid diet without recurrent dysphagia. Lymphocytic esophagitis is a poorly-understood entity. The primary symptom is dysphagia, though symptoms can include chest pain, abdominal pain, and heartburn. A wide range of ages at diagnosis has been reported. Patients often have GERD and atopic comorbidities. Endoscopic findings include rings, esophagitis, strictures, furrows, and nodularity. Biopsies are required for diagnosis and show a high number of intraepithelial lymphocytes but no more than rare neutrophils and eosinophils. Appropriate treatment for LE has not yet been defined, though prognosis appears to be good. This patient's pathology does not meet strict histologic criteria for LE. This may reflect a diagnosis of EoE, an overlap syndrome, or LE with post-surgical changes. LE should be considered in the differential diagnosis of dysphagia and more research is needed on the diagnosis, management, and natural history of this disorder.