PROPKD Score (Renal Survival in ADPKD)

The PROPKD score (Cornec-Le Gall and colleagues, JASN 2016), predicting renal survival in autosomal dominant polycystic kidney disease.

Open the calculator → Runs in your browser. Data leaves only if you deliberately send a problem report from the interactive tool.

Example

Mutation category
Truncating PKD1 mutation
Male sex
Yes
Hypertension before 35 years of age
Yes
First urologic event before 35 years of age
No

Result: PROPKD 7 of 9: high risk.

The tool opens with these values already filled in. Replace them with your own.

What the result means

Mutation
PKD2 = 0, non-truncating PKD1 = 2, truncating PKD1 = 4. Up to 4 of the 9 points.
0 is a FINDING
Scoring PKD2 at 0 asserts it was FOUND. An ungenotyped patient has NO score. No "not tested" option.
0 to 3
Low risk. Median age for ESRD 70.6 years.
4 to 6
Intermediate risk. Median 56.9 years.
7 to 9
High risk. Median 49 years.
Age gate
Both clinical events count only BEFORE age 35, so the score is weakest in young patients.
Scope
Group-level prediction of ESRD age. Not a diagnosis, not current function, not a treatment indication.

What you enter

  • Mutation category.
  • Male sex.
  • Hypertension before 35 years of age.
  • First urologic event before 35 years of age.
  • Optional current age.
Full field descriptions
  • Mutation category. REQUIRED, with NO "unknown" or "not tested" option: scoring PKD2 at 0 points asserts the mutation was FOUND, and an ungenotyped patient has no PROPKD score at all [pkd2 = PKD2 mutation found, 0 points; pkd1-nontruncating = Non-truncating PKD1 mutation, 2 points; pkd1-truncating = Truncating PKD1 mutation, 4 points]
  • Male sex. 1 point.
  • Hypertension before 35 years of age. 2 points.
  • First urologic event before 35 years of age. 2 points. A urologic event means gross hematuria, cyst infection, or flank pain related to cysts. It is not any urological problem.
  • Optional current age. Does NOT enter the score. Used only to flag the documented limitation that the instrument may not identify rapid progression below age 35.
How this is calculated
Weights
Male 1; hypertension before 35 = 2; first urologic event before 35 = 2; mutation 0/2/4.

Cornec-Le Gall E, Audrezet MP, Rousseau A, 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. Read the source ↗

A reference and educational tool. Not medical, legal, or financial advice, and not a substitute for clinician judgment.

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Built by Clay Good. Source on GitHub.