Phospholipase A2 receptor–related membranous nephropathy and mannan-binding lectin deficiency
S Bally, H Debiec, D Ponard, F Dijoud… - Journal of the …, 2016 - journals.lww.com
Most patients with idiopathic membranous nephropathy (IMN) have IgG4 autoantibodies
against phospholipase A2 receptor (PLA2R). C3 and C5b-9 are found in immune deposits of
IMN kidney biopsy specimens, but the pathway of complement activation in IMN remains
elusive. We report the case of a patient who developed IMN with intense staining for PLA2R,
IgG4, C3, C5b-9, factor B, and properdin and very weak staining for C1q, C4d, and IgG1.
Measurement of mannan binding lectin (MBL) antigenic level and activity revealed MBL …
against phospholipase A2 receptor (PLA2R). C3 and C5b-9 are found in immune deposits of
IMN kidney biopsy specimens, but the pathway of complement activation in IMN remains
elusive. We report the case of a patient who developed IMN with intense staining for PLA2R,
IgG4, C3, C5b-9, factor B, and properdin and very weak staining for C1q, C4d, and IgG1.
Measurement of mannan binding lectin (MBL) antigenic level and activity revealed MBL …