Polycystic Kidney Disease Progression and Management

Disease Characteristics

The American Academy of Nephrology notes that most patients with ADPKD experience slow, gradual progression, with kidney failure typically occurring after the fourth decade of life 1
Approximately 3% of children with ADPKD develop very-early-onset or unusually rapid progressive disease, according to the National Institute of Diabetes and Digestive and Kidney Diseases 2
The European Renal Association states that GFR remains nearly always within the normal range during childhood in the majority of cases, making eGFR decline a suitable progression marker only in the small subgroup with very advanced disease 3, 4

The National Kidney Foundation recommends that early-onset hypertension, affecting 6-22% of children, be considered a risk factor for kidney volume and disease progression 5
Proteinuria is one of the most established risk factors for CKD progression in ADPKD, regardless of age, as stated by the American Society of Nephrology 3, 4, 6
Higher urinary sodium levels increase the risk of a composite endpoint of 50% eGFR reduction, ESRD, or death in patients with later-stage ADPKD, according to the International Society of Nephrology 3, 6

The American College of Cardiology recommends that Tolvaptan be used specifically for patients at risk of rapidly progressive disease, demonstrating a net difference in eGFR decline of 1.3 ml/min per 1.73 m² per year compared to untreated patients 7
The European Society of Hypertension states that ACE inhibitors or ARBs should be used as primary treatment in patients with proteinuria, as reduction of proteinuria is associated with significant improvement in renal survival 3, 6