IgA Nephropathy and Atypical Anti-GBM Disease: A Rare Dual Pathology in a Pediatric Rapidly Progressive Glomerulonephritis
Introduction: Anti-GBM nephritis in the pediatric age group is exceedingly rare with concurrent additional pathologies being even rarer. Tissue diagnosis requires a combination of crescentic histomorphology, immunofluorescence showing “paint brush stroke” pattern of linear IgG or rarely IgA, and serum anti-GBM antibodies subject to the disease course and treatment. The authors describe one such case with a dual pathology involving IgA nephropathy and atypical anti-GBM disease. Case Presentation: A 13-year-old girl presenting with features of rapidly progressive glomerulonephritis underwent a renal biopsy showing a mesangioproliferative histology with crescents and an immunofluorescence pattern indicating a dual pathology of IgA nephropathy and anti-GBM nephritis. Additional ancillary testing including staining for IgG subclasses and galactose-deficient IgA (KM55) helped to confirm the diagnosis. She responded to steroid pulses and plasma exchange therapy, was off dialysis after 8 weeks with a serum creatinine level of 1.5 mg/dL, and however remains proteinuric at last follow-up. Conclusion: Concurrent anti-GBM nephritis and IgA nephropathy is a rare occurrence and possibly arises from a complex interaction between the anti-GBM antibodies and the basement membrane unmasking the antigens for IgA antibodies. Additional newer techniques like immunofluorescence for KM55 are helpful in establishing the dual pathology.
- Standort
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Deutsche Nationalbibliothek Frankfurt am Main
- Umfang
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Online-Ressource
- Sprache
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Englisch
- Erschienen in
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IgA Nephropathy and Atypical Anti-GBM Disease: A Rare Dual Pathology in a Pediatric Rapidly Progressive Glomerulonephritis ; volume:2 ; number:1 ; year:2021 ; pages:54-58 ; extent:4
Glomerular Diseases ; 2, Heft 1 (2021), 54-58 (gesamt 4)
- Urheber
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Bajaj, Varun
Thakur, Shilpi
Barwad, Adarsh
Sinha, Aditi
Bagga, Arvind
Singh, Geetika
- DOI
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10.1159/000521582
- URN
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urn:nbn:de:101:1-2022020923314075972994
- Rechteinformation
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Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
- Letzte Aktualisierung
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15.08.2025, 07:24 MESZ
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Beteiligte
- Bajaj, Varun
- Thakur, Shilpi
- Barwad, Adarsh
- Sinha, Aditi
- Bagga, Arvind
- Singh, Geetika
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