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DOI: 10.1055/s-0045-1811211
Perplexing Case of IgG4-Related Mastitis: Hitherto Undescribed Contrast-Enhanced Mammography Features
Funding None.

Abstract
Immunoglobulin G4-related mastitis (IgG4-RM) represents localized form of IgG4-related disease (IgG4-RD), an immune-driven condition marked by abnormal infiltration of plasma cells and raised IgG4 levels. Breast involvement is exceedingly rare and often misinterpreted as malignancy. We report a case of a 40-year-old woman presenting with right axillary lymphadenopathy and clinical suspicion of occult breast malignancy. Contrast-enhanced mammography (CEM) revealed multiple small, equal-density, microlobulated masses in both breasts, exhibiting moderate heterogeneous enhancement. The findings and pattern of involvement favored a secondary breast involvement rather than primary malignancy. 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography demonstrated FDG-avid lesions in the breasts, liver, and spleen, pointing toward systemic immune-mediated pathology. Core biopsy from the breast confirmed IgG4-RM. The patient responded extremely well to oral corticosteroids. This case highlights the diagnostic challenges of IgG4-RM and underscores the critical role of radiologic-pathologic correlation. To the best of our knowledge, this case is the first report in the literature to illustrate the CEM features of IgG4-RM, offering novel imaging insights into this underrecognized entity.
Keywords
IgG4-related mastitis - IgG4-related disease - contrast-enhanced mammography - breast malignancy - pseudotumor - storiform fibrosis* Contributed equally with the first author and shares first authorship.
Publication History
Article published online:
14 August 2025
© 2025. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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