Eculizumab in dense-deposit disease after renal transplantation

Pediatr Nephrol. 2014 Oct;29(10):2055-9. doi: 10.1007/s00467-014-2839-y. Epub 2014 Jun 8.


Background: Dense-deposit disease (DDD) is a rare glomerulopathy characterized by electron-dense deposits in the glomerular basement membrane. About 50 % of patients with DDD progress to end-stage kidney disease and require dialysis within 10 years of diagnosis, and the disease often recurs after renal transplantation.

Case-diagnosis/treatment: We describe a 14-year-old girl with recurrent DDD in her transplanted kidney. Clinical onset was at 8 years of age, when steroid-resistant nephrotic syndrome was diagnosed with microhematuria, severe hypocomplementemia and normal kidney function. Although remission was initially observed after several plasma exchanges, nephrotic proteinuria returned and kidney function further declined 1 year later. The patient received a living-related kidney transplant. Initial allograft function was good, but proteinuria reappeared 3 months after transplantation, accompanied by a slight deterioration in kidney function. After histological confirmation of DDD recurrence and subsequent management with plasmapheresis, the patient was treated for 30 months with eculizumab, a humanized monoclonal antibody that binds to C5 complement protein. This intervention proved effective and resulted in complement inhibition, sustained remission of proteinuria and preservation of renal function. A graft biopsy 6 months later showed no progression of the renal lesions.

Conclusions: Early clinical and histological recurrence of DDD in the transplanted kidney in this 14-year-old patient was treated for 30 months with eculizumab. The patient remains asymptomatic, has no proteinuria and her kidney function is intact.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Antibodies, Monoclonal, Humanized / therapeutic use*
  • Female
  • Glomerulonephritis, Membranoproliferative / drug therapy*
  • Humans
  • Kidney Transplantation*
  • Recurrence


  • Antibodies, Monoclonal, Humanized
  • eculizumab