Department of Radiology, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK.
Department of Renal Medicine, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK.
Clin Radiol. 2023 Aug;78(8):555-564. doi: 10.1016/j.crad.2023.03.003. Epub 2023 Mar 21.
IgG4-related disease is a multisystem immune-mediated disorder associated with lesions manifesting an IgG4-rich plasma cell infiltrate and often raised serum IgG4 concentrations. The disease can mimic neoplastic, infective, and inflammatory processes due to features such as development of masses or organ enlargement. Prompt consideration of this diagnosis is essential to avoid unnecessary investigations and offer appropriate treatments, which can include steroids and other immunosuppressive agents. Although histology is typically diagnostic, imaging is critical to assess disease burden, determine biopsy targets, and evaluate response to treatment. Characteristic imaging features can also point towards the diagnosis in the absence of biopsy. This review highlights these features, as well as more atypical findings, grouped by organ or system. Differential diagnoses are emphasised. The full spectrum of imaging methods is discussed. Whole-body imaging with integrated 2-[F]-fluoro-2-deoxy-d-glucose (FDG) positron-emission tomography (PET)/computed tomography (CT) has an evolving role in the detection of multi-organ involvement and subsequent follow-up.
IgG4 相关疾病是一种多系统免疫介导的疾病，与表现为 IgG4 丰富浆细胞浸润的病变有关，常伴有血清 IgG4 浓度升高。由于肿块或器官肿大等特征，该疾病可能模仿肿瘤、感染和炎症过程。及时考虑这一诊断对于避免不必要的检查和提供适当的治疗至关重要，这些治疗包括类固醇和其他免疫抑制剂。尽管组织学通常具有诊断意义，但影像学对于评估疾病负担、确定活检靶点以及评估治疗反应至关重要。在没有活检的情况下，特征性的影像学特征也可以指向诊断。本综述按器官或系统分组，重点介绍了这些特征以及更不典型的发现。强调了鉴别诊断。讨论了完整的影像学方法。全身成像结合 2-[F]-氟-2-脱氧-D-葡萄糖（FDG）正电子发射断层扫描（PET）/计算机断层扫描（CT）在检测多器官受累和随后的随访方面具有不断发展的作用。
