Zhao Jasmine, Ghani Asmar, Mohammadzadeh Ali, Chan Cato, Shi James
Department of Radiology, University of California Irvine, University of California Irvine Medical Center, Orange, CA, USA.
Radiol Case Rep. 2025 Aug 5;20(10):5347-5352. doi: 10.1016/j.radcr.2025.07.022. eCollection 2025 Oct.
Immunoglobulin G4-related disease (IgG4-RD) is a rare fibroinflammatory disorder with diverse manifestations that can affect nearly any organ system. This case report describes a 58-year-old man presenting with obstructive jaundice due to a biliary stricture concerning for cholangiocarcinoma; however, eventual pathology, serology and imaging findings confirmed IgG4-RD. Over 2 years, the disease progressed to involve multiple organs, including 3-vessel coronary arteritis, a serious but underrecognized manifestation that may present with the ``pigs-in-a-blanket" sign on imaging. Despite treatment, the patient ultimately suffered a fatal cardiac event soon after. This case underscores the importance of recognizing characteristic imaging features, particularly those outlined in the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria, which can act as a framework for radiologists and clinicians in early diagnosis. Prompt recognition, multidisciplinary collaboration, and timely treatment are essential to avoid unnecessary surgical interventions and reduce the risk of life-threatening complications.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种罕见的纤维炎性疾病，表现多样，可累及几乎任何器官系统。本病例报告描述了一名58岁男性，因胆管狭窄导致梗阻性黄疸，怀疑为胆管癌；然而，最终的病理、血清学和影像学检查结果确诊为IgG4-RD。在两年多的时间里，疾病进展累及多个器官，包括三支血管冠状动脉炎，这是一种严重但未得到充分认识的表现，影像学上可能出现“热狗肠”征。尽管进行了治疗，患者最终还是在不久后发生了致命的心脏事件。本病例强调了识别特征性影像学表现的重要性，特别是2019年美国风湿病学会/欧洲抗风湿病联盟分类标准中概述的那些表现，这些表现可为放射科医生和临床医生早期诊断提供框架。及时识别、多学科协作和及时治疗对于避免不必要的手术干预和降低危及生命并发症的风险至关重要。
