Sickle Cell Disease and Renal Complications

Sickle cell disease (SCD) and sickle cell trait have long been associated with a wide extent of renal complications with implications that range from elevated renal plasma flow in young patients to a rapid decline in kidney function and increased mortality in adults. A recent comprehensive study unveiled the intricate link between sickle cell syndromes and kidney abnormalities by highlighting the pressing health concern that affects millions globally. The crucial findings of the study were published in the Nature Reviews Nephrology.

Studies have elucidated the multifactorial pathophysiology underlying SCD-related nephropathy, implicating oxidative stress, hyperfiltration, and glomerular hypertension as key contributing factors. The journey of kidney function in individuals with SCD unfolds a tale of transition with glomerular filtration rates initially increased in youth, which is normalized in early adulthood and eventually plummets to subnormal levels with advancing age.

The prevalence of albuminuria as an early indicator of glomerular damage was observed to afflict a significant portion of individuals with SCD. The kidney function deteriorates at an accelerated pace in SCD patients when compared to individuals with sickle cell trait or healthy counterparts. The genetic modifiers further complicate the landscape of SCD-related nephropathy with variants such as APOL1, HMOX1, HBA1, and HBA2 implicated in disease development and progression. Chronic kidney disease was found as a formidable adversary, and its presence and the rapid decline in estimated glomerular filtration rate serve as ominous predictors of increased mortality among adult SCD patients.

The treatment options for albuminuria in SCD remain somewhat limited, with renin–angiotensin–aldosterone system inhibitors being chosen as the standard of care amidst the lacking conclusive evidence of long-term efficacy. The medical community should remain careful with numerous studies investigating novel therapeutic agents in a bid to reduce the burden of SCD-related kidney complications.

Also, kidney transplantation was observed as a positive hope for individuals with end-stage renal disease due to SCD by underlining the importance of early evaluation and intervention. With kidney disease in SCD continuing to soar, the calls for intensified research efforts reverberate throughout the medical community. Additional studies are imperative to unravel the intricacies of SCD-related nephropathy to delineate its natural history and devise more effective treatment strategies to safeguard the health and well-being of afflicted individuals.

Source

Ataga, K. I., Saraf, S. L., & Derebail, V. K. (2022). The nephropathy of sickle cell trait and sickle cell disease. In Nature Reviews Nephrology (Vol. 18, Issue 6, pp. 361–377). Springer Science and Business Media LLC. https://doi.org/10.1038/s41581-022-00540-9