How can disease progression be predicted in autosomal dominant polycystic kidney disease (ADPKD)?

Updated: Mar 24, 2020
  • Author: Roser Torra, MD, PhD; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Answer

The Mayo Clinic calculator for ADPKD is a useful tool for predicting disease progression. Recommendations for assessing rapid progression of ADPKD have been provided by European experts. [26]

The PROPKD score predicts risk of progression to ESRD in patients with ADPKD. The score is calculated on the basis of the following factors [27] :

  • Male sex: 1 point
  • Hypertension before 35 years of age: 2 points
  • First urologic event before 35 years of age: 2 points
  • PKD2 mutation: 0 points
  • Nontruncating PKD1 mutation: 2 points
  • Truncating PKD1 mutation: 4 points

Risk categories, on the basis of point totals, are as follows:

  • 0-3 points: low risk; median age for ESRD onset 70.6 years
  • 4-6 points: intermediate risk; median age for ESRD onset 56.9 years
  • 7-9 points: high risk; median age for ESRD onset 49 years

A PROPKD score of 3 or less eliminates evolution to ESRD before 60 years of age with a negative predictive value of 81.4%. A score higher than 6 forecasts ESRD onset before 60 years of age with a positive predictive value of 90.9%. [27]

A study by Sato et al in 55 Japanese patients with ADPKD determined that high serum phosphate levels were independently associated with poor renal prognosis. Risk rose with every 1-mg/dL increase (hazard ratio, 6.78; 95% confidence index, 1.94–34.02; P = 0.002). [28]


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