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Use of a B-cell depleting regimen for antifactor H autoantibody-mediated membranoproliferative glomerulonephritis in a paediatric patient

Lookup NU author(s): Professor David KavanaghORCiD

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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).


Abstract

A male child presented initially well with a mixed nephrotic-nephritic syndrome and was commenced on standard high-dose oral corticosteroids. Clinical deterioration occurred 3 weeks later with rapidly progressing renal dysfunction, seizures and diminished urinary output, requiring renal replacement therapy. Once stabilised, renal biopsy demonstrated mesangial and capillary C3, minimal IgG deposition, with mesangial electron dense deposits felt consistent with postinfectious glomerulonephritis or C3 glomerulopathy. Further investigations identified circulating autoantibody directed against factor H, as a plausible aetiology of the membranoproliferative glomerulonephritis (MPGN). Treatment with rituximab and mycophenolate mofetil was associated with a reduction in antibody titres and a concurrent reduction in proteinuria and normalisation of renal function.Subsequent monitoring of antibody titres prompted further administrations of rituximab, with reduction in titres demonstrated after repeat doses. Atypical presentations or complications of nephrotic syndrome or MPGN should prompt detailed investigations for the cause with consideration of antifactor H antibodies.


Publication metadata

Author(s): Henderson S, Ardill R, Reynolds B, Kavanagh D

Publication type: Article

Publication status: Published

Journal: BMJ Case Reports

Year: 2022

Volume: 15

Print publication date: 01/04/2022

Online publication date: 20/04/2022

Acceptance date: 05/04/2022

Date deposited: 26/07/2022

ISSN (electronic): 1757-790X

Publisher: BMJ

URL: https://doi.org/10.1136/bcr-2021-246281

DOI: 10.1136/bcr-2021-246281

ePrints DOI: 10.57711/qerc-2g66

PubMed id: 35444020


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