IgG4-Related Disease
- 24 January 2014
- journal article
- review article
- Published by Annual Reviews in Annual Review Of Pathology-Mechanisms Of Disease
- Vol. 9 (1), 315-347
- https://doi.org/10.1146/annurev-pathol-012513-104708
Abstract
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition that can affect almost any organ and is now being recognized with increasing frequency. IgG4-RD is characterized by a lymphoplasmacytic infiltrate composed of IgG4(+) plasma cells, storiform fibrosis, obliterative phlebitis, and mild to moderate eosinophilia. The diagnosis of IgG4-RD unifies many eponymous fibroinflammatory conditions that had previously been thought to be confined to single organs. IgG4-RD lesions are infiltrated by T helper cells, which likely cause progressive fibrosis and organ damage. IgG4 antibodies are generally regarded as noninflammatory. Although autoreactive IgG4 antibodies are observed in IgG4-RD, there is no evidence that they are directly pathogenic. Rituximab-induced B cell depletion in IgG4-RD leads to rapid clinical and histological improvement accompanied by swift declines in serum IgG4 concentrations. Although IgG autoantibodies against various exocrine gland antigens have been described in IgG4-RD, whether they are members of the IgG4 subclass is unknown. The contribution of autoantibodies to IgG4-RD remains unclear.This publication has 150 references indexed in Scilit:
- B cell depletion therapy ameliorates autoimmune disease through ablation of IL-6-producing B cellsThe Journal of Experimental Medicine, 2012
- B cells and autoimmunityCurrent Opinion in Immunology, 2011
- Autoantibodies Against the Exocrine Pancreas in Autoimmune Pancreatitis: Gene and Protein Expression Profiling and Immunoassays Identify Pancreatic Enzymes as a Major Target of the Inflammatory ProcessThe American Journal of Gastroenterology, 2010
- Macrophages: Master Regulators of Inflammation and FibrosisSeminars in Liver Disease, 2010
- Chimeric IgG4 PR3‐ANCA induces selective inflammatory responses from neutrophils through engagement of Fcγ receptorsImmunology, 2009
- M-Type Phospholipase A2Receptor as Target Antigen in Idiopathic Membranous NephropathyThe New England Journal of Medicine, 2009
- Differential regulation of central nervous system autoimmunity by TH1 and TH17 cellsNature Medicine, 2008
- Eosinophils Act as Antigen‐Presenting Cells to Induce Immunity toStrongyloides stercoralisin MiceThe Journal of Infectious Diseases, 2007
- CD4+CD25+Foxp3+regulatory T cells induce alternative activation of human monocytes/macrophagesProceedings of the National Academy of Sciences of the United States of America, 2007
- Follicular B helper T cells in antibody responses and autoimmunityNature Reviews Immunology, 2005