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Pediatric Nephrology

, Volume 29, Issue 11, pp 2225–2228 | Cite as

Eculizumab treatment for rescue of renal function in IgA nephropathy

  • Therese Rosenblad
  • Johan Rebetz
  • Martin Johansson
  • Zivile Békássy
  • Lisa Sartz
  • Diana KarpmanEmail author
Brief Report

Abstract

Background

Immunoglobulin A (IgA) nephropathy is a chronic glomerulonephritis with excessive glomerular deposition of IgA1, C3 and C5b-9, which may lead to renal failure.

Case Diagnosis/Treatment

We describe the clinical course of an adolescent with rapidly progressive disease leading to renal failure in spite of immunosuppressive treatment. Due to refractory disease the patient was treated with eculizumab (anti-C5) for 3 months in an attempt to rescue renal function. Treatment led to clinical improvement with stabilization of the glomerular filtration rate and reduced proteinuria. Discontinuation of treatment led to a rapid deterioration of renal function. This was followed by a single dose of eculizumab, which again reduced creatinine levels temporarily.

Conclusions

Early initiation of eculizumab therapy in patients with progressive IgA nephropathy may have a beneficial effect by blocking complement-mediated renal inflammation.

Keywords

IgA nephropathy Complement Eculizumab 

Notes

Financial support

This study was supported by the Swedish Research Council (Grant K2013-64X-14008), The Torsten Söderberg Foundation, Crown Princess Lovisa’s Society for Child Care, The Konung Gustaf V:s 80-årsfond (all to DK). MJ is supported by the Marianne & Marcus Wallenberg Foundation, The Swedish Kidney Disease Association and Malmö University Hospital Research Funds.

Disclosures

Diana Karpman was the national coordinator in Sweden of the international trial of eculizumab (Alexion Pharmaceuticals) during 2009–2010. Lisa Sartz is the current national coordinator of the Alexion aHUS registry in Sweden.

Supplementary material

467_2014_2863_MOESM1_ESM.pptx (1.3 mb)
Supplementary Figure 1 (PPTX 1296 kb)

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Copyright information

© IPNA 2014

Authors and Affiliations

  • Therese Rosenblad
    • 1
  • Johan Rebetz
    • 2
  • Martin Johansson
    • 3
    • 4
  • Zivile Békássy
    • 1
  • Lisa Sartz
    • 1
  • Diana Karpman
    • 1
    • 2
    Email author
  1. 1.Section of Pediatric Nephrology, Department of Pediatric and Adolescent MedicineSkåne University HospitalLundSweden
  2. 2.Department of Pediatrics, Clinical Sciences LundLund UniversityLundSweden
  3. 3.Department of PathologySkåne University HospitalMalmöSweden
  4. 4.Department of Laboratory Medicine Malmö, Center for Molecular PathologyLund UniversityMalmöSweden

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