title: IgG4 antibodies in autoimmune polyglandular disease and IgG4-related endocrinopathies: pathophysiology and clinical characteristics.
abstract: OBJECTIVE
  This review discusses the IgG4-related disease spectrum (IgG4-RD), the autoimmune polyglandular syndromes (APS), the association of IgG4 with APS, and possible pathobiology.
  
  
  RESULTS
  IgG4-RD is a multiorgan autoimmune disorder characterized by fibrous inflammation, IgG4-positive plasma cell infiltration in affected tissues, and elevated serum concentrations of IgG4. IgG4-RD can affect any organ and has a heterogeneous presentation. Consensus criteria for diagnosis in specific organs have been established. The recognition and diagnosis of IgG4-RD are crucial because the disease responds favorably to immunosuppression (e.g., glucocorticoids, rituximab). The precise mechanisms leading to disease are unknown, but IgG4 antibodies may undergo a half antibody exchange, which renders them incapable of activating the complement pathway.
  
  
  CONCLUSIONS
  Despite significant advances in disease recognition and treatment strategies, the disorder remains poorly understood. The precise role of IgG4, whether it is protective or pathogenic, is still being debated.
authors.affiliation: aDepartment of Pediatrics, Division of Pediatric Endocrinology bDepartments of Pathology, Immunology and Laboratory Medicine, Pediatrics, and Molecular Genetics and Microbiology, University of Florida, Gainesville, Florida, USA.
authors.forename: Bimota
authors.initials: B
authors.lastname: Nambam
authors.forename: William E
authors.initials: WE
authors.lastname: Winter
authors.forename: Desmond A
authors.initials: DA
authors.lastname: Schatz
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001323:Autoantibodies / Q000097:blood; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007074:Immunoglobulin G / Q000187:drug effects / Q000276:immunology*; D007155:Immunologic Factors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007249:Inflammation / Q000188:drug therapy / Q000276:immunology*; D016884:Polyendocrinopathies, Autoimmune / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab