Department of Radiology and Biomedical Imaging, Yale School of Medicine, 333 Cedar Street, Room TE-2, PO Box 208042, New Haven, CT, 06520, USA.
Diagnostic Radiology Resident, Yale-New Haven Hospital, New Haven, CT, USA.
Abdom Radiol (NY). 2023 Oct;48(10):3147-3161. doi: 10.1007/s00261-023-03996-w. Epub 2023 Jul 6.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated disease that can affect multiple organs, including the orbits, salivary glands, thyroid gland, lungs, aorta, pancreas, bile ducts, lymph nodes, and retroperitoneum. While timely diagnosis is particularly important given the efficacy of glucocorticoid treatment for IgG4-RD, accurate recognition can prove a challenge given the overlap between the imaging features of this disease and other entities.
After a review of the epidemiology, pathophysiology, and clinical considerations (including treatment) associated with IgG4-RD, this pictorial review will showcase the variable imaging manifestations of this disease in the abdomen and pelvis. Post-treatment imaging appearance of these entities will be reviewed and mimickers of this disease in the abdomen and pelvis will be presented.
The presence of mass-like soft tissue with radiographic characteristics of fibrosis affecting multiple organs should raise suspicion for IgG4-RD, although definite diagnosis can only be made with appropriate clinical, serological, and pathologic data.
免疫球蛋白 G4 相关疾病（IgG4-RD）是一种全身性、免疫介导的疾病，可影响多个器官，包括眼眶、唾液腺、甲状腺、肺、主动脉、胰腺、胆管、淋巴结和腹膜后腔。鉴于 IgG4-RD 的糖皮质激素治疗效果显著，及时诊断尤为重要，但鉴于该病的影像学特征与其他实体之间存在重叠，准确识别可能具有挑战性。
在回顾 IgG4-RD 相关的流行病学、病理生理学和临床注意事项（包括治疗）后，本影像学综述将展示该病在腹部和骨盆中的各种影像学表现。我们将回顾这些病变的治疗后影像学表现，并介绍腹部和骨盆中该病的类似病变。
存在累及多个器官的具有纤维化为放射学特征的块状软组织时，应怀疑 IgG4-RD，但只有结合适当的临床、血清学和病理学数据，才能做出明确诊断。
