Jeremy is 29 years old and has been diagnosed with ADPKD by a kidney ultrasound. Jeremy is at risk for rapid disease progression, as indicated by the following risk factors: being male, urologic events before age 35, and family history of ESKD by age 58. Let's take a closer look with some prognostic tools to confirm the risk of rapid disease progression and assess how rapidly his disease may progress.1,2

  • Age29
  • Height6'1"
  • Weight179 lbs
  • SexM
  • Race (AA/O)AA

Baseline Assessment2-6

  • Serum creatinine (mg/dL)1.21
  • eGFR (mL/min/1.73m2)80.4
  • Ultrasound kidney length (cm)Not obtained
  • Hypertension before 35?Not yet observed
  • Urological event before 35?Yes
  • Family members with ESKD?Yes (50 yrs)
  • MutationsNot available
  • PROPKD ScoreNot available
  • htTKV (mL/m)Perform MRI/CT
  • Other risk factorsNone
  • ADPKD Imaging ClassificationMRI or CT needed

Disease Progression2,3,5-10

Normal htTKV range: 150-250Years1720232629323538414447mL/m0500100015002000
eGFR: 80.4 mL/min/1.73m2
CKD 1CKD 2CKD 3CKD 4CKD 5/ESRDYears1720232629323538414447mL/min/1.73m²0102030405060708090100


Click on the flags below to walk through an assessment that confirms risk of rapid disease progression and assesses how rapidly Jeremy’s disease may progress

ADPKD=autosomal dominant polycystic kidney disease; ESKD=end stage kidney disease; AA=African American; O=other; eGFR=estimated glomerular filtration rate; PKD=polycystic kidney disease; htTKV=height-adjusted total kidney volume; MRI=magnetic resonance imaging; CT=computed tomography; CKD=chronic kidney disease.

1. Schrier RW, et al. Predictors of autosomal dominant polycystic kidney disease progression. J Am Soc Nephrol. 2014;25:2399-2418.

2. Gansevoort RT, et al. Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice. Nephrol Dial Transplant. 2016;31(3):337-48.

3. Wetzels JFM, et al. Age- and gender-specific reference values of estimated GFR in caucasians: the Nijmegen biomedical study. Kidney Int. 2007;72:632-637.

4. Cornec-Le Gall E, et al. The PROPKD score: a new algorithm to predict renal survival in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2016;27(3):942-951.

5. Irazabal MV, et al. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol. 2015;26:160-172.

6. Imaging classification of ADPKD: a simple model for selecting patients for clinical trials. Accessed January 09, 2019.

7. Cheong B, et al. Normal values for renal length and volume as measured by magnetic resonance imaging. Clin J Am Soc Nephrol. 2007;2:38-45.

8. Levey AS, et al. Definition and classification of chronic kidney disease: a position statement from kidney disease: improving global outcomes (KDIGO). Kidney Int. 2005;67:2089-2100.

9. PKD Charity. Fast Facts about ADPKD. The Polycystic Kidney Disease Charity. 2017. Accessed January 09, 2019.

10. Rangan GK, et al. Autosomal dominant polycystic kidney disease: a path forward. Semin Nephrol. 2015;35(6):524-537.

The ADPKDsim website is intended for healthcare professionals and for educational purposes only. The tool and content on this site are not meant to be a substitute for medical judgement and should not be used for diagnosis and/or treatment decisions. Healthcare professionals should use independent judgment when considering these educational resources.