Prognostic Tools

Clinical Risk Factors and Prognostic Tools

There are a number of clinical risk factors associated with rapid disease progression in ADPKD. If one or more of these risk factors are present, different prognostic tools can be used to confirm the risk of rapid progression. Some of these prognostic tools can also assess how rapidly the disease may progress and even estimate time to ESRD.^1-3

Select a tool below to learn more.

MRI/CT-calculated Total Kidney Volume

Clinical Risk Factors:

TKV greater than expected for age

How it works:

Uses simple MRI or CT kidney measurements to calculate TKV and determine low, intermediate or high risk for eGFR decline.

When is it appropriate?

For patients who have access to MRI or CT.^4,5

PROPKD Score

Clinical Risk Factors:

Being male
Hypertension before age 35
Urologic events before age 35
Truncating
PKD1 mutation

How it works:

Tallies four risk factors to determine low, intermediate, or high risk for progression to ESRD.

When is it appropriate?

For patients who have genetic test results.^6,7

ERA-EDTA Algorithm

Clinical Risk Factors:

Rapid historical eGFR decline*
Family history of ESRD by age 58

How it works:

Hierarchically evaluates risk factors to confirm likelihood of rapid disease progression.

When is it appropriate?

For patients with a variety of risk factors.^2,7

Ultrasound Kidney Length

Clinical Risk Factors:

US-KL > 16.5 cm in patients less than 45 years of age†

How it works:

Can predict rapid disease progression in patients less than 45 years old.

When is it appropriate?

When MRI/CT-calculated TKV is not available.^3,7

*Confirmed eGFR decline ≥ 5 mL/min/1.73 m² within a year and/or ≥ 2.5 mL/min/1.73 m² per year over a period of five years.

†When MRI-/CT-calculated TKV is not available, US-KL may be a useful surrogate when rapid progression is defined as CKD stage 3 development within 8 years^2,3

ADPKD=autosomal dominant polycystic kidney disease; ESRD=end-stage renal disease; eGFR=estimated glomerular filtration rate; US-KL=ultrasound kidney length; PKD=polycystic kidney disease; TKV=total kidney volume; MRI=magnetic resonance imaging; CT=computed tomography; ERA-EDTA=European Renal Association – European Dialysis and Transplant Association.

References:

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. Nephrol Dial Transplant. 2016;31:337-348.

3. Bhutani H, et al. A comparison of ultrasound and magnetic resonance imaging shows that kidney length predicts chronic kidney disease in autosomal dominant polycystic kidney disease. Kidney In. 2015;88:146-151.

4. Higashihara E, et al. Kidney volume estimations with ellipsoid equations by magnetic resonance imaging in autosomal dominant polycystic kidney disease. Nephron. 2015;129:253-262.

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. 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.

7. Chebib FT, et al. J Am Soc Nephrol. 2018;29(10):2458-2470.