title: IgG4-related diseases.
abstract: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a fascinating condition recognised as a systemic disease in 2003 [1,2]. The first link between autoimmunity affecting the pancreas, elevated serum IgG4 concentrations and large numbers of IgG4-positive plasma cells in pancreatic tissue was described only 2 years earlier [3]. Since then, many diseases that have long been viewed organ-specific are now considered within the spectrum of IgG4-RD. Practically any organ can be affected, having in common a key pathological feature consisting in dense lymphocyte and plasma cell infiltrate rich in IgG4-positive plasma cells, storiform fibrosis and often an elevated serum IgG4 concentration. While good clinical response to steroid therapy is observed, immunosuppressive or B-cell depleting therapy can be required. It is important to distinguish the IgG4-RD from traditional organ-specific autoimmune disease to guide therapy.
authors.affiliation: Division of Rheumatology, Allergy and Immunology, UC San Diego, School of Medicine, La Jolla, CA, USA. mguma@ucsd.edu
authors.forename: Monica
authors.initials: M
authors.lastname: Guma
authors.forename: Gary S
authors.initials: GS
authors.lastname: Firestein
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D009928:Organ Specificity / Q000276:immunology; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D035583:Rare Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D012737:Sex Factors
chemical_list: D007074:Immunoglobulin G; D007166:Immunosuppressive Agents