ArticlePediatric nephrology (Berlin, Germany)2017
PLA2R-positive (primary) membranous nephropathy in a child with IPEX syndrome.
Article in Pediatric nephrology (Berlin, Germany), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 14 citations in OpenAlex.
- Inborn errors of regulatory T-cell differentiation and function.The Journal of allergy and clinical immunology · 2025Review
- Clinicopathologic features of non-lupus membranous nephropathy in a pediatric population.Pediatric nephrology (Berlin, Germany) · 2022Review
- A novel FOXP3 mutation in a Chinese child with IPEX-associated membranous nephropathy.Molecular genetics & genomic medicine · 2022Review
- Non-lupus full-house nephropathy-immune dysregulation as a rare cause of pediatric nephrotic syndrome: Answers.Pediatric nephrology (Berlin, Germany) · 2022Article
- Kidney involvement in a child with autoimmune disease: Answers.Pediatric nephrology (Berlin, Germany) · 2021Article
- Renal Involvement in IPEX Syndrome With a Novel Mutation ofFrontiers in genetics · 2021Article
- Atypical Presentations of IPEX: Expect the Unexpected.Frontiers in pediatrics · 2021Review
- How Times Have Changed! A Cornucopia of Antigens for Membranous Nephropathy.Frontiers in immunology · 2021Review
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Authors and funding
6 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundImmunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare primary immunodeficiency syndrome characterized by the development of multiple autoimmune disorders in affected individuals. Different forms of renal injury have been reported in IPEX syndrome, and membranous nephropathy (MN) is among the most common glomerulopathies found. However, the exact pathogenesis of MN in this setting has not been elucidated, and it is not clear whether it is part of the clinical spectrum of the disease or secondary to medications, infections or other concomitant insults. DIAGNOSIS/TREATMENT: We describe a child diagnosed with IPEX syndrome shortly after birth who presented with nephrotic syndrome at the age of 11 weeks. Renal biopsy revealed a MN with enhanced immunohistochemical staining for phospholipase A2 receptor (PLA2R).
conclusionThis is the first report of a PLA2R-positive MN in a patient with IPEX syndrome. We suggest that, in this context, MN results from an autoimmune process against podocytic antigens, namely PLA2R.
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