[Spectrum of renal manifestations in sickle cell disease]

Sickle cell disease (SCD), the most common hemoglobinopathy, is an increasing cause of chronic kidney disease. In the last decade, we have witnessed a better understanding in the characterization of clinical manifestations and pathogenesis of sickle cell nephropathy. The spectrum of renal diseases during SCD includes various renal manifestations such as impairment of urinary concentrating ability, defect in urine acidification, renal papillary necrosis and proteinuria related to glomerular injury leading to progressive end-stage renal disease. Endothelial dysfunction related to chronic hemolysis and the relative renal hypoxia caused by vaso-occlusive sickle red blood cells are probably two key factors for SCN development. Optimal therapeutic management (including the use of blockers of the renin-angiotensin system) of patients with proteinuria remains to be determined. Renal replacement therapy with dialysis is required in SCD patients with end-stage renal disease but these patients should probably undergo kidney transplantation that requires careful management.