Abstract：
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.
摘要：
免疫球蛋白G4相关疾病（IgG4-RD）是一种日益公认的导致炎症的免疫介导疾病，狭窄和质量形成。根据所涉及的器官系统，它会引起广泛的疾病和临床表现。孤立的食管IgG4-RD很少见，诊断可能很困难。它对皮质类固醇反应强烈，早期识别和激励管理是关键。我们描述了一名47岁男子的病例，该男子在进行性吞咽困难和体重减轻的背景下出现食团阻塞。影像学和胃镜检查显示弥漫性食管增厚，并出现良性狭窄。在活检和非诊断性内窥镜超声引导细针抽吸的非特异性组织学发现后，他接受了电视胸腔镜手术和食管穿刺活检。这证实了IgG4-RD的诊断。最初的治疗是皮质类固醇。然而,由于皮质类固醇断奶后症状复发，开始硫唑嘌呤维持治疗。硫唑嘌呤先前已用于IgG4-RD的全身性病例，但尚未报道用于孤立的食管疾病。该病例突出了食管IgG4-RD的诊断和治疗困难，并且当组织学显示食管炎伴淋巴浆细胞浸润时，需要将其视为鉴别诊断。
