IgG4-Related Systemic Disease
- 1 January 2012
- journal article
- research article
- Published by Ovid Technologies (Wolters Kluwer Health) in Medicine
- Vol. 91 (1), 49-56
- https://doi.org/10.1097/md.0b013e3182433d77
Abstract
IgG4-related systemic disease is now recognized as a systemic disease that may affect various organs. The diagnosis is usually made in patients who present with elevated IgG4 in serum and tissue infiltration of diseased organs by numerous IgG4+ plasma cells, in the absence of validated diagnosis criteria. We report the clinical, laboratory, and histologic characteristics of 25 patients from a French nationwide cohort. We also report the treatment outcome and show that despite the efficacy of corticosteroids, a second-line treatment is frequently necessary. The clinical findings in our patients are not different from the results of previous reports from Eastern countries. Our laboratory and histologic findings, however, suggest, at least in some patients, a more broad polyclonal B cell activation than the skewed IgG4 switch previously reported. These observations strongly suggest the implication of a T-cell dependent B-cell polyclonal activation in IgG4-related systemic disease, probably at least in part under the control of T helper follicular cells.Keywords
This publication has 21 references indexed in Scilit:
- Rituximab for the Treatment of IgG4-Related DiseaseMedicine, 2012
- Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related diseaseThe Esophagus, 2011
- Pathogenesis of IgG4-related diseaseCurrent Opinion in Rheumatology, 2011
- Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritisKidney International, 2010
- Possible involvement of T helper type 2 responses to Toll-like receptor ligands in IgG4-related sclerosing diseaseGut, 2010
- Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disordersAnnals Of The Rheumatic Diseases, 2008
- Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan-Korea Symposium on Autoimmune PancreatitisThe Esophagus, 2008
- The Sensitivity and Specificity of Serum Immunoglobulin G and Immunoglobulin G4 Levels in the Diagnosis of Autoimmune Chronic PancreatitisPancreas, 2007
- Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitisHepatology, 2007
- Clinical diagnostic criteria of autoimmune pancreatitis: revised proposalThe Esophagus, 2006