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

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Case Reports: Rare disease
Use of a B-cell depleting regimen for antifactor H autoantibody-mediated membranoproliferative glomerulonephritis in a paediatric patient
  1. Sarah Henderson1,
  2. Rosalyn Ardill1,
  3. Ben Reynolds2,3 and
  4. David Kavanagh4,5
  1. 1Paediatric Renal Department, NHS Lothian, Edinburgh, UK
  2. 2Paediatric Renal Department, NHS Greater Glasgow and Clyde, Glasgow, UK
  3. 3Department of Child Life and Health, University of Glasgow, Glasgow, UK
  4. 4Complement Therapeutics Research Group, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK
  5. 5National Renal Complement Therapeutics Centre, Newcastle Upon Tyne Hospitals NHS Trust, Newcastle Upon Tyne, UK
  1. Correspondence to Dr Sarah Henderson; sarah.henderson{at}nhslothian.scot.nhs.uk

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.

  • Paediatrics
  • Nephrotic syndrome
  • Renal medicine

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

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    Footnotes

    • Contributors BR provided clinical care for the patient and initiated the manuscript. RA provided clinical care for the patient and drafted the manuscript. DK drafted the manuscript, created the figure on complement activation and regulation and performed specialist investigations. SH drafted and prepared all versions of the manuscript and the rest of the figures. All authors contributed to and approve the final version.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.