ESPN 50th Annual Meeting

ESPN 2017


 
Successful kidney allograft in patient with Dense Deposit Disease
DELBET JEAN-DANIEL 1 ULINSKI TIM 1 AUGER MARTIN 1

1- PEDIATRIC NEPHROLOGY UNIT, ARMAND-TROUSSEAU HOSPITAL, AP-HP, UNIVERSITY PARIS 6, UPMC, PARIS, FRANCE
 
Introduction:

To describe a case of successful kidney transplant in a patient with Dense-deposit- disease (DDD), without recurrence of the initial pathology after prophylactic treatment with eculuzimab.

Material and methods:

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. The disease often recurs post-transplant. Some clinical reports show an efficacity of eculizumab traitement, a humanized monoclonal antibody that binds to C5 complement protein.

Results:

We describe an 18-year-old girl with a successful kidney allograft for end-stage renal disease due to DDD. At the age of 9 years she was diagnosed with steroid resistant nephrotic syndrome associated to microscopic hematuria, severe hypocomplementemia and renal failure. Kidney biopsy revealed DDD. Despite treatment with steroids and mycophenolate mofetil (MMF), the kidney function further declined over 2 years, and hemodialysis was started at the age of 14 years. She received a cadaveric  transplant at 17 years and we chose an immunosuppressive treatment including one IV steroid pulse of 500 mg/m2, oral steroids over 3 months, tacrolimus, MMF and eculizumab (8 infusions over the first 2 months post-transplant). Biological follow up was done with weekly complement C3 level analysis and proteinuria. There was complete terminal complement pathway inhibitionno proteinuria recurred and eculizumab treatment was discontinued after 2 months. Protocol kidney biopsies were performed at M1, 4, and 11 without signs of disease recurrence. Graft function remained normal and proteinuria negative after eculizumab discontinuation.

Conclusions:

Eculizumab might be an interesting tool to prevent disease recurrence post-transplant in patients with DDD. Careful monitoring of histological and biological parameters during and in particular after eculizumab treatment are necessary to reduce the potential interval between disease recurrence and eculizumab (re)-treatment.