IgG4-related disease
SYSTEMIC FIBROINFLAMMATORY DISEASE, ASSOCIATED WITH ELEVATED SERUM IGG4 LEVELS IN ABOUT 60% OF CASES
Mikulicz's disease; Hyper-IgG4 disease; IgG4–related systemic disease; IgG4-related systemic disease; IgG4-RD; Immunoglobulin G4-related disease
IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and a usually prompt response to oral steroids. In approximately 51–70% of people with this disease, serum IgG4 concentrations are elevated during an acute phase.