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Recurrence of Complement Factor H-Related Protein 5 Nephropathy in a Renal Transplant

2010; Elsevier BV; Volume: 11; Issue: 1 Linguagem: Inglês

10.1111/j.1600-6143.2010.03333.x

1600-6143

Katherine A. Vernon, Daniel P. Gale, Elena Goicoechea de Jorge, A. McLean, Jack Galliford, Alkis Pierides, Patrick H. Maxwell, David Taube, Matthew C. Pickering, H. Terence Cook,

Coagulation, Bradykinin, Polyphosphates, and Angioedema

Complement factor H-related protein 5 (CFHR5) nephropathy is a familial renal disease endemic in Cyprus. It is characterized by persistent microscopic hematuria, synpharyngitic macroscopic hematuria and progressive renal impairment. Isolated glomerular accumulation of complement component 3 (C3) is typical with variable degrees of glomerular inflammation. Affected individuals have a heterozygous internal duplication in the CFHR5 gene, although the mechanism through which this mutation results in renal disease is not understood. Notably, the risk of progressive renal failure in this condition is higher in males than females. We report the first documented case of recurrence of CFHR5 nephropathy in a renal transplant in a 53-year-old Cypriot male. Strikingly, histological changes of CFHR5 nephropathy were evident in the donor kidney 46 days post-transplantation. This unique case demonstrates that renal-derived CFHR5 protein cannot prevent the development of CFHR5 nephropathy.