>ERA-EDTA Algorithm

European Renal Association − European Dialysis and Transplant Association position statement - ERA-EDTA Algorithm

This algorithm hierarchically evaluates risk factors in order of reliability to determine likelihood of rapid progression.¹

CKD stage by age:

at age 18-30 yr: CKD 1-3a (eGFR > 45 mL/min/1.73 m²)

at age 30-40 yr: CKD 2-3a (eGFR 45-90 mL/min/1.73 m²)

at age 40-50 yr: CKD 3a (eGFR 45-60 mL/min/1.73 m²)

No: Likely slow progression

Yes: Proceed to next step

Historical eGFR decline, with no other confounding cause than ADPKD:

1) confirmed eGFR decline ≥ 5 mL/min/1.73 m² in one year and/or

2) confirmed eGFR decline ≥ 2.5 mL/min/1.73 m² per year over a period of five years or more?

No: Likely slow progression

Data not available or not reliable
(e.g. in CKD 1)

Yes: Rapid progression

Historical kidney growth in typical ADKPD:

(ht)TKV lncrease more than 5 % per year by repeated measurements (≥ 3)?

Preferable by MRI (ellipsoid equation), if not available then by another reliable method (CT)

No: Likely slow progression

Data not available or not reliable

Yes: Rapid progression

Predicted progression by baseline htTKV indexed for age and/or genotype:

1) htTKV compatible with ADPKD Imaging Class 1C, 1D, 1E or US length > 16.5 cm and/or

2) truncating PKD1 mutation + early symptoms (i.e. a PROPKD score > 6)?

No: Likely slow progression

Data not available or not reliable

Yes: Likely rapid progression

Predicted progression by baseline htTKV indexed for age and/or genotype:

1) htTKV compatible with ADPKD Imaging Class 1C, 1D, 1E or US length > 16.5 cm and/or

2) truncating PKD1 mutation + early symptoms (i.e. a PROPKD score > 6)?

No: Likely slow progression

Yes: Possible rapid progression

Adapted with permission of Oxford University Press, from Gansevoort RT, et al. Nephrol Dial Transplant. 2016;31(3):337-348.

Limitations

The premise that eGFR indexed for age above high cut off values can distinguish rapidly from slowly progressive disease in the majority of patients with ADPKD is not accurate.²
Inclusion by rapid historical eGFR decline may not account for high variability of eGFR values > 60 mL/min/1.73 m² and historical factors affecting historical values often unavailable.²
Serial TKV measurements may not be practical.²

ERA-EDTA=European Renal Association – European Dialysis and Transplant Association; eGFR=estimated glomerular filtration rate; CKD=chronic kidney disease; ADPKD=autosomal dominant polycystic kidney disease; htTKV=height-adjusted total kidney volume; MRI=magnetic resonance imaging; CT=computed tomography; US=ultrasound; PKD=polycystic kidney disease; ESKD=end-stage kidney disease.

References:

1. 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.

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

The information provided on the ADPKDsim website is intended for your educational benefit only. It is not intended as, nor is it a substitute for medical care or advice or professional diagnosis.

Health care professionals should use their independent judgment when reviewing the ADPKDsim's educational resources. Users seeking medical advice should consult with a health care professional.