%0 Journal Article
%T Isolated esophageal IgG4-related disease presenting with progressive dysphagia and weight loss in a middle-aged man.
%A Poole H
%A Fehily S
%A McKelvie P
%A Miller A
%A Lust M
%A Holmes JA
%A Poole H
%A Fehily S
%A McKelvie P
%A Miller A
%A Lust M
%A Holmes JA
%J Clin J Gastroenterol
%V 15
%N 3
%D Jun 2022
%M 35381953
暂无%R 10.1007/s12328-022-01623-7
%X Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized immune-mediated condition that results in inflammation, stricturing and mass formation. It causes a wide spectrum of disease and clinical presentations depending on the organ system involved. Isolated esophageal IgG4-RD is rare and diagnosis can be difficult. It is highly responsive to corticosteroids, and early identification and instigation of management is key.We describe the case of a 47-year-old man who presented with a food bolus obstruction on a background of progressive dysphagia and weight loss. Imaging and gastroscopy demonstrated diffuse esophageal thickening with a benign appearing stricture. Following non-specific histologic findings on biopsy and a non-diagnostic endoscopic ultrasound guided fine needle aspiration, he underwent video-assisted thoracoscopic surgery with esophageal core biopsy. This confirmed the diagnosis of IgG4-RD. Initial treatment was with corticosteroids. However, due to recurrence of symptoms upon weaning of corticosteroids, azathioprine maintenance therapy was instituted. Azathioprine has previously been used in systemic cases of IgG4-RD but has not been reported for isolated esophageal disease.This case highlights the difficulties in the diagnosis and treatment of esophageal IgG4-RD and the need to consider it as a differential diagnosis when histology reveals esophagitis with lymphoplasmacytic infiltration.