Pathology of IgG4-related sclerosing mastitis

Immunoglobulin G4-related sclerosing mastitis (IgG4-RM) is a recently recognised member of the IgG4-related disease (IgG4-RD) family, a multisystem fibroinflammatory condition that can affect any organ system. IgG4-RM is rare and predominantly occurs in middle-aged women. It may present with painless palpable mass and/or lymphadenopathy thereby mimicking breast cancer. Although there is an abundance of literature describing the clinicopathological characteristics of IgG4-RD in a variety of organs, data on IgG4-RM are limited due to its rarity. This review describes the manifestation of the disease in the breast based on reported cases, emphasising the clinicopathological features, pathophysiology, differential diagnosis, treatment and prognosis.