The kidney in sickle hemoglobinopathies
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With improvements in the care of patients with sickle hemoglobinopathies, sickle cell disease (SCD) has evolved from a disease that was fatal in childhood into one in which most survive past their 5th decade and some into old age. As a result, the renal complications of sickle hemoglobinopathies, which are age dependent, have emerged as a common and serious complication of SCD. Approximately 14 - 18% of mortality in SCD is attributed to chronic kidney disease (CKD), which develops in 1/3 of individuals with SCD and progresses to end-stage renal disease in 4 - 18% of them. Importantly, the presence of CKD increases the risk of the other systemic complications of SCD, with the median survival of SCD estimated at 51 years, declining to 29 years in those with CKD. The obstructive vasculopathy of SCD affects the glomerulus, tubules, and medulla of the kidney. Albuminuria and inability to concentrate the urine precede the onset of renal failure, and, along with other tubular dysfunctions, are early warning signs of sickle cell nephropathy (SCN). This is a review of the historical background SCN, the pathophysiology of the renal lesions, their varied clinical and pathologic manifestations, and available treatment options.
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