Genetic variants in membranous nephropathy: perhaps a perfect storm rather than a straightforward conformeropathy?

DJ Salant - Journal of the American Society of Nephrology, 2013 - journals.lww.com
Primary (or idiopathic) membranous nephropathy is an organ-specific autoimmune disease
in which circulating antibodies to a conformation-dependent epitope in the target antigen,
the M-type receptor for phospholipase A2 (PLA2R), are detectable in up to 80% of patients
from various ethnic groups. 1, 2 It mainly affects older adults and is unusual in that the
immune deposits and circulating anti-PLA2R antibodies are predominantly IgG4. Primary
membranous nephropathy is the most common cause of nephrotic syndrome in white …