Phospholipase A2 receptor staining in pediatric idiopathic membranous glomerulopathy
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.
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.
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.
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.
KeywordsPhospholipase A2 receptor PLA2R Membranous glomerulonephritis Membranous nephropathy Membranous glomerulopathy IgG4
The authors thank Robert Yerton, Steve Strong, and Cindy Smith for their valuable technical aid and I-Shen Wen and Tina Priddy for their administrative support. The abstract to this work was presented at the USCAP 102nd Annual Meeting (2013), Baltimore, MD, USA (Mod Pathol 26: 383–396; doi: 10.1038/modpathol.2013.16).
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