Sickle cell disease and the kidney

The renal features of sickle cell disease include some of the most common reasons for referral to nephrologists. This author, from the University of Kansas Medical Center, comprehensively reviews the renal effects of sickle cell disease, including hematuria, papillary necrosis, tubular disturbances and chronic glomerulopathy. All aspects of these complications are covered, including their presentation, pathogenesis, pathology, risk factors, diagnosis and treatment. The renal features of sickle cell disease (SCD) include some of the most common reasons for referral to nephrologists, such as hematuria, proteinuria, tubular disturbances and chronic kidney disease. Therapy of these conditions requires specialized knowledge of their distinct pathogenic mechanisms. Painless hematuria is usually benign—unless massive—and can be treated with hydration alone if renal medullary carcinoma has been ruled out. Tubular functional defects, which tend to reduce urinary concentrating capacity, generally require no specific treatment. Proteinuria might indicate the development of chronic sickle cell nephropathy, which can be treated effectively. Measurement of glomerular filtration rate in SCD is problematic, which makes identification and monitoring of chronic kidney disease difficult in patients with SCD. Although managing and predicting the outcomes of chronic kidney disease in the SCD setting is challenging, affected individuals do benefit from transplantation. This Review summarizes the presentation, processes, pathology, modifiers, diagnosis and treatment of the renal effects of SCD. Key Points Hematuria in sickle cell disease (SCD) or sickle trait can be benign and usually requires only conservative management and an awareness of more-serious potential diagnoses such as renal medullary carcinoma The principal tubule disorder in SCD is defective urinary concentrating capacity, but this complication should not generate serious problems unless there is injudicious use of fluids, NSAIDs or antihypertensive medications, or renal insufficiency Chronic sickle cell nephropathy is a progressive form of focal segmental glomerulosclerosis that seems to begin with hyperperfusion and microalbuminuria and is probably amenable to measures to prevent its progression Transplantation is a good option for patients with advanced sickle cell nephropathy, although the outcomes are not as favorable as for other types of end-stage renal disease The measurement and monitoring of glomerular f