IgG4‐Related disease is not associated with antibody to the phospholipase A2 receptor

A Khosroshahi, R Ayalon, LH Beck Jr… - International journal …, 2012 - Wiley Online Library
International journal of rheumatology, 2012Wiley Online Library
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 …
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.
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