PS01.097: A CASE OF IGG4-RELATED SCLEROSING ESOPHAGITIS

Abstract Background IgG4-related disease (IgG4-RD) is a systemic disease characterized by fibrosing inflammation with an abundance of IgG4-positive plasma cells. IgG4-RD has been found to affect various organs. Many cases of IgG4-RD affecting the extrapancreatic organs have been reported in patients without pancreatic lesions. Methods Sclerosing esophagitis with IgG4-positive plasma cell infiltration is very rare disease. We report a case of esophageal benign stricture that was difficult to diagonose. Results A 76-year-old woman had been experiencing slight difficulty in swallowing in the 3 years prior to this presentation. Her dysphagia had progressed and she began to vomit frequently after meals and lost weight while hospitalized after surgery for cervical cancer. Esophagogastroduodenoscopy and an esophagram showed circumferential erosion and a stricture of the thoracic esophagus. No malignant cells were identified. However, because it was difficult to exclude a malignant tumor, she underwent esophageal resection. The resected specimen showed stricture with mucosal erosion and transmural wall hypertrophy. On histological examination, transmural hyperplasia consisting of inflammatory granulation tissue with abundant infiltration of IgG4-positive plasma cells and lymphocytes was observed. Conclusion A probable diagnosis of IgG4-related disease was made because comprehensive diagnostic criteria for this condition were met. IgG4-related esophageal disease presenting as esophageal lesions alone is very rare. Disclosure All authors have declared no conflicts of interest.