Immunoglobulin G4-related disease (
IgG4-RD) is a newly recognized systemic
syndrome characterized by elevated
serum IgG4 concentrations and tumefaction or
tissue infiltration by
IgG4-positive
plasma cells. We experienced a case of
IgG4-RD involving multiple organs in a 64-year-old
female who was referred for a suspected uroepithelial
tumor. A mass
biopsy confirmed dense lymphoplasmacytic infiltration with an increased number of
IgG4-positive
plasma cells. We discuss this case and
review the
literature to bring
IgG4-RD to the
attention to clinicians because it responds dramatically well to
steroid therapy and should be kept in mind as a
differential diagnosis to avoid
unnecessary surgery.