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Immunoglobulin G4-Related Disease (IgG4-RD) Presenting As Multiple Myeloma

IgG4-RD is a rare syndrome with various presentations including eosinophilia, elevated serum IgG4, organ swelling and lymphadenopathy.

The etiology of IgG4-RD is unknown and requires a tissue biopsy typically showing extensive lymphocyte and plasma cells infiltration, IgG4 immunoblasts, storiform fibrosis (fibrosis in a cartwheel distribution), eosinophil tissue infiltration and obliterative phlebitis. Elevated serum IgG4 levels or eosinophilia may be present.
Treatment with systemic glucocorticosteroids is recommended in symptomatic and asymptomatic cases involving kidney or pancreas.