IgG4-related disease is not associated with antibody to the phospholipase A2 receptor

• Type of Material: Article
• Authors: Arezou Khosroshahi, Emory University; Rivka Ayalon, Boston University School of Medicine; Laurence H. Beck, Jr., Boston University School of Medicine; David J. Salant, Boston University School of Medicine; Donald B. Bloch, Massachusetts General Hospital; John H. Stone, Massachusetts General Hospital
• Language: English
• Date: 2012-06-20
• Publisher: International Journal of Rheumatology
• Publication Version: Final Published Version
• Copyright Statement: © 2012 Arezou Khosroshahi et al.
• License: Creative Commons Attribution 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1155/2012/139409
• Title of Journal or Parent Work: International Journal of Rheumatology
• Volume: 2012
• Start Page: 139409
• End Page: 139409
• Grant/Funding Information: The analysis of anti-PLA2R antibodies was supported by National Institutes of Health research Grants DK030932 and DK090029 (to DJS) and by a Career Development Grant from the Halpin Foundation-American Society of Nephrology (LHB).
• Keywords: IgG4-related disease (IgG4-RD)
• Research Categories: Health Sciences, Pathology

Abstract

Patients with IgG4-related disease (IgG4-RD) share histopathological characteristics that are similar across affected organs. The finding of infiltration with IgG4+ plasma cells in the proper clinical and histopathological contexts connects a large number of clinical entities that were viewed previously as separate conditions. The renal involvement in IgG4-RD is usually characterized by tubulointerstitial nephritis, but membranous nephropathy has also been reported to be one of the renal complications of IgG4-RD. The recent discovery that a high proportion of patients with idiopathic membranous nephropathy (IMN) have IgG4 autoantibodies to the M-type phospholipase A2 receptor (PLA2R) in the circulation and glomerular immune deposits, together with the profound IgG4 hypergammaglobulinemia and occasional reports of membranous nephropathy in IgG4-RD, raised the question of a common antigen. To assess the presence of anti-PLA2R antibody in patients with IgG4-RD, we screened sera from 28 IgG4-RD patients by immunoblot. None of the patients in this cohort had detectable circulating anti-PLA2R antibodies. This study suggests that despite some clinical and serological overlaps between IgG4-RD and IMN,anti-PLA2R antibodies do not play a role in the pathogenesis of IgG4-RD. Additional studies of IgG4-RD with evidence of membranous nephropathy are important to exclude any definite relationship.