AJKD Atlas of Renal Pathology: Sickle Cell Nephropathy

Sickle cell disease is an autosomal recessive disease that occurs most commonly in individuals of African ancestry, with an incidence of 1 in 500 African Americans. Kidney disease has varying manifestations, with microalbuminuria in childhood, progressing to overt proteinuria in 20%-25% and nephrotic syndrome in about 5%, and progressive GFR loss after age 20 years in 5%-8% of all sickle cell patients. Patients often show hyposthenuria and hematuria as well. Some patients develop papillary necrosis and acute kidney injury due to sickle cell crisis.