title: Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation: C3-Glomerulonephritis as an Initial Presentation.
abstract: Risk for atypical hemolytic uremic syndrome (aHUS) recurrence after renal transplantation is low with an isolated membrane cofactor protein mutation (MCP). We report the case of a 32-year-old woman with a MCP who underwent kidney transplantation with a good evolution at 12 months. At 15 and 35 months, 2 episodes of thrombotic microangiopathy (TMA), after a miscarriage and a preeclampsia, were misinterpreted as triggered by tacrolimus. After each episode however serum creatinine returned to baseline. Five years after transplantation, she had a self-limited rhinosinusitis followed 3 weeks later by an oliguric renal failure. Her complement profile was normal. Graft biopsy showed C3 glomerulonephritis with no "humps" on electron microscopy. No significant renal function improvement followed methylprednisolone pulsing. A second biopsy showed severe acute TMA lesions with C3 glomerular deposits. Despite weekly eculizumab for 1 month, dialysis was resumed. A new workup identified the "at-risk" complement factor H haplotype. Thus, aHUS recurrence should be ruled out in aHUS patients considered at low recurrence risk when a TMA is found in graft biopsy. Prompt eculizumab therapy should be considered to avoid graft loss as aHUS recurrence can first present as a C3 glomerulonephritis.
authors: Division of Nephrology, Geneva University Hospitals, Geneva, Switzerland.
authors: Yassine
authors: Y
authors: Bouatou
authors: Laboratoire d'immunologie biologique, Hôpital Européen Georges-Pompidou, Assistance publique-Hôpitaux de Paris, Paris, France.
authors: Véronique Frémeaux
authors: VF
authors: Bacchi
authors: Immunology and Transplant Unit, Service of Immunology and Allergology, Geneva University Hospitals, Geneva, Switzerland.
authors: Jean
authors: J
authors: Villard
authors: Institute of Clinical Pathology, Geneva University Hospitals, Geneva, Switzerland.
authors: Solange
authors: S
authors: Moll
authors: Division of Nephrology, Geneva University Hospitals, Geneva, Switzerland.
authors: Pierre-Yves
authors: PY
authors: Martin
authors: Division of Nephrology, Geneva University Hospitals, Geneva, Switzerland.; Division of Transplantation, Geneva University Hospitals, Geneva, Switzerland.
authors: Karine
authors: K
authors: Hadaya