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Updated: Jul 12, 2025
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Sickle Cell Disease and CKD: An Update
Rima S Zahr1, Santosh L Saraf2
1Division of Pediatric Nephrology and Hypertension, University of Tennessee Health Science Center, Memphis, Tennessee, USA.
Insights
Sickle cell disease commonly affects kidneys, leading to early damage like loss of urine concentration and hyperfiltration. Targeted therapies and research are crucial for improving kidney health and survival in affected individuals.
Area of Science:
- Nephrology
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) affects millions globally, causing organ damage through vaso-occlusion and hemolysis.
- Kidneys are frequently impacted in SCD, leading to significant morbidity.
Purpose of the Study:
- To summarize the renal manifestations and management of sickle cell disease-related kidney damage.
- To highlight genetic risk factors and current therapeutic limitations.
Main Methods:
- Literature review of renal complications in sickle cell disease.
- Analysis of genetic associations (APOL1, HMOX1, α-thalassemia) with sickle nephropathy.
- Evaluation of current treatment strategies and transplantation outcomes.
Main Results:
- Early kidney damage includes medullary ischemia, impaired urine concentration, and hyperfiltration.
- Progressive damage involves albuminuria and declining glomerular filtration rate.
- APOL1 variants increase sickle nephropathy risk, while α-thalassemia may offer protection.
Conclusions:
- Kidney disease significantly contributes to mortality in sickle cell disease patients.
- Current therapies have limited efficacy data; novel treatments are needed.
- Kidney transplantation improves survival but is underutilized.
Background:
Sickle cell disease is an inherited red blood cell disorder that affects approximately 100,000 people in the USA and 25 million people worldwide. Vaso-occlusion and chronic hemolysis lead to dysfunction of vital organ systems, with the kidneys being among the most commonly affected organs.
Summary:
Early renal manifestations include medullary ischemia with the loss of urine-concentrating ability and hyperfiltration. This can be followed by progressive damage characterized by persistent albuminuria and a decline in the estimated glomerular filtration rate. The risk of sickle nephropathy is greater in those with the APOL1 G1 and G2 kidney risk variants and variants in HMOX1 and lower in those that coinherit α-thalassemia. Therapies to treat sickle cell disease-related kidney damage focus on sickle cell disease-modifying therapies (e.g., hydroxyurea) or those adopted from the nonsickle cell disease kidney literature (e.g., renin-angiotensin-aldosterone system inhibitors), although data on their clinical efficacy are limited to small studies with short follow-up periods. Kidney transplantation for end-stage kidney disease improves survival compared to hemodialysis but is underutilized in this patient population.
Key Messages:
Kidney disease is a major contributor to early mortality, and more research is needed to understand the pathophysiology and develop targeted therapies to improve kidney health in sickle cell disease.
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