ERA-EDTA Algorithm

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

eGFR indexed for age

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
Rapid historical eGFR decline

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
Rapid historical eGFR decline

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 rapid progression by ADPKD Imaging Class or PROPKD

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 rapid progression by ADPKD Imaging Class or PROPKD

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
ERA-EDTA Algorithm Flowchart