PROPKD Score

Predicting Renal Outcome in Polycystic Kidney Disease (PROPKD) Scoring system predicts the likely hood of kidney failure before 60 years old.

Multivariate survival analysis identified four variables that were significantly associated with age at ESKD onset, and a scoring system from 0 to 9 was developed as follows:¹

PROPKD Calculator

being male

hypertension before 35 years of age

first urologic event* before 35 years of age

mutation

PKD2 mutation

nontruncating PKD1 mutation

^§truncating PKD1 mutation

PROPKD Score =

SUM

Sample PROPKD Score Calculation

ADPKD patient info: 29 year old male with hypertension and a truncating PKD1 mutation

1 point for being male

2 points for hypertension before 35 years of age

4 points for a truncating PKD1 mutation

7 points PROPKD Score

HIGH Risk of Progression to ESKD

LOW

70.6 median age for ESKD onset

Eliminates evolution to ESKD before age 60^†

^†negative predictive value of 81.4%

INTERMEDIATE

56.9 median age for ESKD onset

HIGH

49 median age for ESKD onset

Risk of rapid progression and a 92% chance of reaching kidney failure before age 60.

Adapted with permission of The American Society of Nephrology, from 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.

Limitations

PROPKD score may not be helpful identifying rapid progression in patients < 35 years old unless they are already hypertensive and have experienced urologic complications.²
PROPKD score requires genetic testing.¹
The genetic-based scoring systems are shown to be accurate prognosis for ADPKD patients on a subpopulation level but precision is yet to be demonstrated on an individual level.

*previous urological events defined as gross hematuria, cyst infections, and flank pain related to cysts¹
^§up to 18% of truncating mutations lead to mild, not severe disease

ESKD=end-stage kidney disease; PKD=polycystic kidney disease; ADPKD=autosomal dominant polycystic kidney disease.

References:

1. 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.

2. Chebib FT, Perrone RD, Chapman AB, Dahl NK, Harris PC, Mrug M, Mustafa RA, Rastogi A, Watnick T, Alan SL, Torres VE. A practical guide for treatment of rapidly progressive ADPKD with tolvaptan.Journal of the American Society of Nephrology. 2018; 29(10):2458-70.