St. Joseph's University Medical Center, Paterson, NJ, USA.
St. George's University School of Medicine, Grenada, West Indies.
J Investig Med High Impact Case Rep. 2024 Jan-Dec;12:23247096241286368. doi: 10.1177/23247096241286368.
Immunoglobulin G4-related disease (IgG4-RD) is a multiorgan, fibro-inflammatory condition that presents with painless organ swelling, lymphoplasmacytic infiltration, and obliterative phlebitis, often showing a favorable response to corticosteroid therapy. The most affected organs include the pancreas, kidneys, retroperitoneum, lacrimal glands, and salivary glands. Diagnosis relies on serological, imaging, and histopathological findings, with glucocorticoids as the primary treatment. Despite its reversible nature and good prognosis in many cases, long-term complications such as organ dysfunction or malignancy can still occur. International collaborative efforts have enhanced the understanding, diagnosis, and management of IgG4-RD, emphasizing the importance of comprehensive diagnostic criteria and appropriate therapeutic strategies. Herein, we present an interesting case of a geriatric male who was referred to our clinic because of concern for pancreatic cancer. We diagnosed the patient with autoimmune pancreatitis, a manifestation of IgG4-RD. The patient experienced a dramatic response to steroid therapy and is currently on maintenance therapy.
免疫球蛋白 G4 相关疾病（IgG4-RD）是一种多器官、纤维炎症性疾病，表现为无痛性器官肿胀、淋巴浆细胞浸润和闭塞性静脉炎，常对皮质类固醇治疗有良好反应。最常受累的器官包括胰腺、肾脏、腹膜后腔、泪腺和唾液腺。诊断依赖于血清学、影像学和组织病理学发现，糖皮质激素是主要治疗方法。尽管在许多情况下该病具有可逆转性和良好的预后，但仍可能发生长期并发症，如器官功能障碍或恶性肿瘤。国际合作努力增强了对 IgG4-RD 的认识、诊断和管理，强调了综合诊断标准和适当治疗策略的重要性。在此，我们报告了 1 例老年男性病例，因担心胰腺癌而被转至我科。我们诊断该患者为自身免疫性胰腺炎，这是 IgG4-RD 的一种表现。该患者对类固醇治疗有显著反应，目前正在维持治疗中。
