IgG4-related disease (IgG4-RD) is characterised by high serum concentrations of
IgG4, dense lymphoplasmacytic infiltrates, storiform
fibrosis and increased IgG4-positive plasma cells in tissues. This systemic disease occurs in various organs metachronously, but IgG4-related
mastitis appears extremely rare. We report a case of IgG4-related
mastitis, radiologically considered to represent
breast cancer mainly composed of intraductal component and requiring histological differentiation from mucosa-associated lymphoid tissue (
MALT) lymphoma. The breast mass disappeared with
steroid therapy. When patients have a breast mass, regardless of the presence or absence of
IgG4-RD, IgG4-related
mastitis should be considered in addition to
breast cancer. If histological findings show dense lymphoplasmacytic infiltrates, IgG4-related
mastitis should be suspected in addition to
malignant lymphoma, and lack of monoclonality should be confirmed. To avoid unnecessary surgery or
chemotherapy, knowledge and accurate diagnosis of the entity of IgG4-related
mastitis is necessary.