title: Esophageal Involvement of Immunoglobulin G4-Related Disease: A Case Report and Literature Review.
abstract: Immunoglobulin G4 (IgG4)-related disease is characterized by the typical histopathological features of a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, a high ratio of IgG4- to IgG-positive cells, storiform fibrosis (cellular fibrosis organized in an irregular whorled pattern), obliterative phlebitis, and variable presence of eosinophils. The disease exhibits systemic involvement but very rarely involves the esophagus. A 33-year-old man was admitted to our hospital for evaluation of a 1-year history of progressive dysphagia. Neck imaging revealed a 3.9-cm mass in the cervical esophagus and multifocal calcified lymph nodes in the lower neck and mediastinum. Two previous tertiary hospitals failed to diagnose the patient's condition despite the use of ultrasound-guided needle biopsy of the neck tumor. We performed neck imaging studies, a flexible endoscopic swallowing study, high-resolution manometry, upper endoscopy, and a review of the previous pathologic slides. The patient was finally diagnosed with IgG4-related esophagitis and showed a good response to corticosteroid therapy. We herein report a rare case of dysphagia associated with IgG4-related disease and present a review of the literature.
authors.affiliation: From the Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, College of Medicine, Soonchunhyang University, Seoul (JHO, THL, HSK, CSJ, CSJ, JSL), Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, College of Medicine, Soonchunhyang University, Bucheon (SJH), and Department of Pathology, College of Medicine, Soonchunhyang University, Seoul, Republic of Korea (SYJ).
authors.forename: Ji Hyun
authors.initials: JH
authors.lastname: Oh
authors.forename: Tae Hee
authors.initials: TH
authors.lastname: Lee
authors.forename: Hyo Shik
authors.initials: HS
authors.lastname: Kim
authors.forename: Chan Sung
authors.initials: CS
authors.lastname: Jung
authors.forename: Joon Seong
authors.initials: JS
authors.lastname: Lee
authors.forename: Su Jin
authors.initials: SJ
authors.lastname: Hong
authors.forename: So-Young
authors.initials: SY
authors.lastname: Jin
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D004941:Esophagitis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D014463:Ultrasonography
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G