The glomerulopathy of sickle cell disease

Am J Hematol. 2014 Sep;89(9):907-14. doi: 10.1002/ajh.23762. Epub 2014 Jun 19.


Sickle cell disease (SCD) produces many structural and functional abnormalities in the kidney, including glomerular abnormalities. Albuminuria is the most common manifestation of glomerular damage, with a prevalence between 26 and 68% in adult patients. The pathophysiology of albuminuria in SCD is likely multifactorial, with contributions from hyperfiltration, glomerular hypertension, ischemia-reperfusion injury, oxidative stress, decreased nitric oxide (NO) bioavailability, and endothelial dysfunction. Although its natural history in SCD remains inadequately defined, albuminuria is associated with increased echocardiography-derived tricuspid regurgitant jet velocity, systemic blood pressure, and hypertension, as well as history of stroke, suggesting a shared vasculopathic pathophysiology. While most patients with albuminuria are treated with angiotensin converting enzyme inhibitors/angiotensin receptor blockers, there are no published long-term data on the efficacy of these agents. With the improved patient survival following kidney transplantation, SCD patients with end-stage renal disease should be considered for this treatment modality. Given the high prevalence of albuminuria and its association with multiple SCD-related clinical complications, additional studies are needed to answer several clinically important questions in a bid to adequately elucidate its pathophysiology, natural history, and treatment.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Albuminuria / drug therapy
  • Albuminuria / etiology*
  • Albuminuria / pathology
  • Albuminuria / urine
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / drug therapy
  • Anemia, Sickle Cell / pathology
  • Anemia, Sickle Cell / urine
  • Angiotensin Receptor Antagonists / administration & dosage
  • Angiotensin Receptor Antagonists / therapeutic use
  • Angiotensin-Converting Enzyme Inhibitors / administration & dosage
  • Angiotensin-Converting Enzyme Inhibitors / therapeutic use
  • Glomerulonephritis / drug therapy
  • Glomerulonephritis / etiology*
  • Glomerulonephritis / pathology
  • Glomerulonephritis / urine
  • Humans
  • Kidney Failure, Chronic / drug therapy
  • Kidney Failure, Chronic / etiology*
  • Kidney Failure, Chronic / pathology
  • Kidney Failure, Chronic / urine
  • Kidney Transplantation


  • Angiotensin Receptor Antagonists
  • Angiotensin-Converting Enzyme Inhibitors