IgG4-Related Esophagitis

Abstract

Purpose: IgG4-related disease is an emerging condition characterized by dense lymphocytic inflammation consisting of IgG4 plasma cells and storiform fibrosis, affecting multiple organs, but rarely the upper GI tract. We present two patients with IgG4 esophagitis: one who developed an esophageal stricture and the other who developed esophagitis dissecans and then invasive squamous cell carcinoma. The latter is the first report of IgG4 esophagitis associated with malignancy. Case 1: An 87 year-old female presented to our motility clinic with progressive dysphagia to solids for 1 year that was refractory to sucralfate, H2 blockers and PPI. Upper endoscopy showed an esophageal stricture at 27cm that was subsequently pneumatically dilated. Biopsy of the stricture showed ulcerative esophagitis with an underlying dense chronic inflammation. Immunohistochemical staining revealed a focal population of IgG4-positive plasma cells in the lamina propria, numbering up to 25/HPF. The patient was started on swallow inhaled fluticasone, which provided minimal relief, and repeat biopsy after three months showed increased inflammation with 50 IgG4-positive plasma cells/HPF. Over two years, the patient underwent 12 stricture dilations that provided only temporary relief. Case 2: A 65 year-old man presented to our clinic with worsening dysphagia and odynophagia. Endoscopy showed diffuse narrowing of the esophagus with friable mucosa that easily peeled away with minimal trauma. Biopsy showed diffuse lymphocytic esophagitis with a focal plasma cell infiltrate in the lamina propria of the proximal esophagus. The region of plasma cell infiltrate correlated with esophageal wall thickening seen on endoscopic ultrasound. Staining showed polyclonal plasma cell infiltrate with mostly IgG4-positive plasma cells. The patient was started on steroids, but 6 months later returned with worsening symptoms. Repeat endoscopy revealed an esophageal stricture at 20cm and proximal esophagitis dissecans, as evidenced by sloughing of the esophageal mucosa with minimal trauma. Soon after, the patient developed white lesions on his tongue and buccal mucosa and was diagnosed with cicatricaial pemphigoid. However, this diagnosis was lifted after a subsequent endoscopy showed an invasive squamous cell carcinoma at the proximal esophagus. His tumor was moderately differentiated, invading through the muscularis propria and into the adventita. The patient underwent an esophagogastrectomy thereafter.Figure 1Figure 2