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Multimodality pictorial review of IgG4-related disease in the abdomen and pelvis

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Abstract

Background

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.

Purpose

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.

Conclusion

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.

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Authors and Affiliations

  1. Department of Radiology and Biomedical Imaging, Yale School of Medicine, 333 Cedar Street, Room TE-2, PO Box 208042, New Haven, CT, 06520, USA

    Suzanne Czerniak & Mahan Mathur

  2. Diagnostic Radiology Resident, Yale-New Haven Hospital, New Haven, CT, USA

    Aditya Rao

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  1. Suzanne Czerniak
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Correspondence to Suzanne Czerniak.

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Czerniak, S., Rao, A. & Mathur, M. Multimodality pictorial review of IgG4-related disease in the abdomen and pelvis. Abdom Radiol 48, 3147–3161 (2023). https://doi.org/10.1007/s00261-023-03996-w

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