Department of Gastroenterology, Gastrointestinal Oncology, and Endocrinology, University Medical Center Goettingen, Goettingen, Germany.
Institut of Pathology, University Medical Center Goettingen, Goettingen, Germany.
Am J Case Rep. 2021 May 30;22:e930729. doi: 10.12659/AJCR.930729.
BACKGROUND Immunoglobulin (Ig) G4-related disease is a rare disease of unknown pathophysiology, which can affect multiple organs leading to tissue fibrosis and organ failure. The present case report describes a patient with systemic IgG4-related disease (IgG4-RD) that occurred over a 1-year period and affected multiple organs at different times. Imaging studies, interventional procedures, changes in laboratory parameters, and histopathology demonstrate the novel and known aspects of this disease before and during prednisolone monotherapy and in combination with azathioprine. CASE REPORT A 64-year-old man presented with weight loss and painless jaundice, which was highly suspicious for cholangiocarcinoma. A thorough medical history together with laboratory tests, imaging procedures, and endoscopic interventions confirmed that surgery was not needed and led to the final diagnosis of histologically-confirmed, IgG4-related sclerosing cholangitis and autoimmune pancreatitis type 1. Other typical organ manifestations of systemic IgG4-RD were diagnosed through a thorough medical review, which led to immunohistochemical reevaluation of past surgical specimens. Besides the IgG4-related organ manifestations, which can include periorbital xanthelasmas, our patient developed a pulmonary adenocarcinoma 6 years after the initial clinical onset of IgG4-RD. After immunosuppressive treatment with prednisolone alone and subsequently in combination with azathioprine, the patient's IgG4-RD resolved. CONCLUSIONS Interdisciplinary collaboration is required to diagnose IgG4-RD that involves multiple organs. Patient medical history remains crucial for diagnosis and attention should be paid to avoiding unnecessary surgery. Tumors (lung adenocarcinomas) and xanthelasmas can develop because of IgG4-RD. Glucocorticoids and additional azathioprine may be advisable for maintenance treatment.
免疫球蛋白（Ig）G4 相关疾病是一种病因不明的罕见疾病，可影响多个器官，导致组织纤维化和器官衰竭。本病例报告描述了一名患者，其系统性 IgG4 相关疾病（IgG4-RD）在 1 年内反复发作，不同时间影响多个器官。影像学研究、介入操作、实验室参数变化和组织病理学在泼尼松单药治疗及与硫唑嘌呤联合治疗前后均显示出该疾病的新发病理特征和已知特征。
一名 64 岁男性因体重减轻和无痛性黄疸就诊，高度怀疑为胆管癌。详细的病史、实验室检查、影像学检查和内镜介入检查证实无需手术，并最终诊断为组织学证实的 IgG4 相关硬化性胆管炎和 1 型自身免疫性胰腺炎。通过全面的医学回顾诊断出其他典型的系统性 IgG4-RD 器官表现，这导致对过去手术标本进行免疫组织化学重新评估。除了 IgG4 相关器官表现，包括眶周黄色瘤外，我们的患者在 IgG4-RD 初始临床发病后 6 年还发生了肺腺癌。在单独使用泼尼松和随后联合使用硫唑嘌呤进行免疫抑制治疗后，患者的 IgG4-RD 得到缓解。
需要多学科合作来诊断涉及多个器官的 IgG4-RD。患者的病史对诊断仍然至关重要，应注意避免不必要的手术。由于 IgG4-RD 可导致肿瘤（肺腺癌）和黄色瘤的发生。对于维持治疗，糖皮质激素和额外的硫唑嘌呤可能是明智的选择。
