Phospholipase A2 receptor staining in pediatric idiopathic membranous glomerulopathy

Abstract

Background

Membranous glomerulopathy, though typically a disease of adults, does occur in children. Antiphospholipase A2 receptor (PLA2R) autoantibodies have recently been implicated as a causative agent in most cases of adult primary (idiopathic) membranous glomerulopathy. PLA2R staining of renal biopsies in two recent large case series of adults with primary membranous glomerulopathy showed a sensitivity of approximately 75 % for detecting primary membranous glomerulopathy. To our knowledge, this is the largest study of its kind to assess PLA2R staining in a pediatric population.

Methods

Forty-one consecutive cases of pediatric membranous glomerulopathy were identified from our database, and clinical follow-up was performed to confirm primary membranous glomerulopathy. Twenty-two patients met inclusion criteria and are the subject of this report.

Results

Granular, capillary loop immunofluorescence staining for immunoglobulin G (IgG) was present in 100 % of patients, and C3 staining was present in 77 %. PLA2R staining was identified in ten patients, providing a sensitivity of 45 % [confidence interval (CI) 25–67 %]. Bovine serum albumin staining was performed in all PLA2R-negative cases and showed no positivity. Morphologic findings associated with negative PLA2R staining included segmental membranous lesions, mesangial and subendothelial deposits, C1q and “full-house” staining, and lower-stage lesions by electron microscopy. At 38 months’ average follow-up, all patients were still considered as having primary membranous glomerulopathy, with none developing a clinically detectable secondary etiology.

Conclusions

PLA2R staining sensitivity is much lower in the pediatric than the adult primary membranous glomerulopathy population. This finding suggests a more diverse and currently incompletely described set of etiologies for this disease in this group.