- end-stage renal disease will occur in up to 50$ of patients by 60 years of age. There is great variability in the age of onset of renal failure (2 years to 80 years).
- renal disease appears to have a less aggressive course in women.
- hypertension is also associated with a more aggressive course.
- predictors of more rapid progression to renal failure include the following:
- earlier age at diagnosis
- male sex
- sickle cell trait
- PKD1 genotype
- larger or rapidly increasing kidney size
- gross hematuria
- being black
- increasing proteinuria
- most lethal extrarenal manifestations of ADPKD are intracranial aneurysms,
which has been found to be present in up to 40% of ADPKD patients
- these aneurysms can rupture, causing intracranial hemorrhage and death
in 8% to 11% of patients
- these aneurysms can rupture, causing intracranial hemorrhage and death in 8% to 11% of patients
- risk for renal cancer in ADPKD with renal failure
- incidence of clinically significant renal cell carcinoma (RCC) in ADPKD patients with renal failure is not increased as compared to patients with other kidney diseases, although in some studies removed ADPKD kidneys revealed a 5% to 8% incidence of RCC, most measuring <=2 cm in diameter
- Halvorson CR et al. Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment.Int J Nephrol Renovasc Dis. 2010; 3: 69-83.
- Chapman AB et al. Autosomal Dominant Polycystic Kidney Disease (ADPKD): Executive Summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference.Kidney Int. 2015 Jul; 88(1): 17-27.
Last edited 07/2018 and last reviewed 07/2018