Phospholipase A2 receptor staining in pediatric idiopathic membranous glomerulopathy

Cossey, L. Nicholas; Walker, Patrick D.; Larsen, Chris

doi:10.1007/s00467-013-2574-9

Cited by 45 publications

(28 citation statements)

References 19 publications

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“…The results of the present study are similar to our adult PMN experience. In the study by Cossey et al 7 , 45% of the paediatric PMN cases had enhanced staining for PLA 2 R. Different study population may explain the difference. There was a very good association of aPLA 2 R with clinical response.…”

Section: Discussionmentioning

confidence: 92%

“…The study included 11 (61.11%) male and seven (38.89%) female subjects. The duration of illness was 10.11 ± 5.26 (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19) months. The mean proteinuria, serum albumin and serum creatinine was 4.52 ± 1.93 (2.43-9.20) g/day, 1.99 ± 0.68 (1.04-3.40) g/dL and 1.04 ± 1.67 (Median 0.70, IQR 0.48-0.82) mg/dL.…”

Section: Baseline Parametersmentioning

confidence: 99%

“…In our previous report, we observed aPLA2R and enhanced glomerular staining for PLA 2 R in 60 and 100% of childhood onset PMN cases. 6 Cossey et al, 7 in their study of 22 paediatric PMN cases, (adolescent, n-21 (95.45%)) showed enhanced glomerular staining for PLA 2 R in 45% and negative PLA 2 R staining was associated with mesangial expansion and subendothelial deposits, C1q and "full house" staining. None of the cases were positive for BSA staining.…”

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confidence: 96%

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Primary membranous nephropathy in adolescence: A prospective study

et al. 2017

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Section: Discussionmentioning

confidence: 92%

Section: Baseline Parametersmentioning

confidence: 99%

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confidence: 96%

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Primary membranous nephropathy in adolescence: A prospective study

et al. 2017

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“…The ability to stain archival biopsy specimens for PLA 2 R has allowed an analysis of the frequency of PLA 2 R-associated disease in a pediatric population. A recent analysis in biopsies from cases ranging in age from 4 to 17 has shown that 10/22 (45%) of pediatric biopsies exhibited PLA 2 R staining in a granular capillary loop pattern; the youngest case was from a ten year-old [49]. Those cases negative for PLA 2 R had more evidence of secondary features, such as mesangial deposits, the presence of C1q, and predominance of an IgG subclass other than IgG4.…”

Section: Pla2r-associated Mnmentioning

confidence: 99%

Membranous nephropathy: not just a disease for adults

Ayalon

Beck

2013

Pediatr Nephrol

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Membranous nephropathy (MN) is an immune complex-mediated cause of the nephrotic syndrome that can occur in all age groups, from infants to the elderly. While systemic disorders such as hepatitis B infection or lupus may more frequently cause secondary MN in the younger population, primary or “idiopathic” MN has generally been considered a disease of adults. Recent progress in our understanding of primary disease was recently made when the target antigen in primary MN was identified as the M-type phospholipase A2 receptor (PLA2R). Circulating anti-PLA2R antibodies may serve both as a diagnostic tool for distinguishing primary from secondary disease, and as a biomarker for monitoring the immunological activity of this organ-specific autoimmune disease during treatment. Whereas definitive therapy for secondary forms of MN should be targeted at the underlying cause, immunosuppressive therapy is often necessary for primary disease. Alkylating agents in combination with corticosteroids, as well as calcineurin inhibitors (+/− steroids), are first line agents due to randomized controlled trials in an adult population with relatively long durations of follow-up. However, rituximab, mycophenolate and ACTH have shown promise in smaller and/or observational studies. The optimal therapy for children and adolescents with MN is less well defined.

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“…Cossey et al . evaluated staining for PLA2R in glomerular deposits in 22 patients with IMN between the ages of 4 and 17 years, and noted positivity in 45% of cases . The frequency of anti‐PLA2R positivity in the paediatric population has not been studied.…”

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confidence: 99%

Antibodies to m‐type phospholipase A2 receptor in children with idiopathic membranous nephropathy

et al. 2015

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Idiopathic membranous nephropathy (IMN), the commonest cause of adult nephrotic syndrome (NS), accounts for only a minority of paediatric NS. Antibodies to m-type phospholipase A2 receptor (PLA2R) are seen in two-thirds of adult IMN cases. PLA2R staining in glomerular deposits is observed in 74% and 45% of adult and paediatric IMN cases, respectively. However, there are no reports of anti-PLA2R in paediatric IMN. We evaluated anti-PLA2R levels and PLA2R in gloemrular deposits in paediatric IMN seen at our center. Five cases were enrolled, all the cases stained for PLA2R in glomeruli and three (60%) had antibodies to PLA2R antigen. There was a parellel reduction in proteinuria and anti-PLA2R titer. The present report suggests that PLA2R has a contributory role in the pathogenesis of paediatric IMN.

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Phospholipase A2 receptor staining in pediatric idiopathic membranous glomerulopathy

Abstract: 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.

Cited by 45 publications

References 19 publications

Primary membranous nephropathy in adolescence: A prospective study

Primary membranous nephropathy in adolescence: A prospective study

Membranous nephropathy: not just a disease for adults

Antibodies to m‐type phospholipase A2 receptor in children with idiopathic membranous nephropathy

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