Risk Prediction Using PROPKD Score
Cornec-Le Gall et al developed the PROPKD score as a prognostic model to predict renal outcomes in patients with ADPKD on the basis of genetic and clinical data from 1341 patients from the Genkyst cohort.50 The scoring system assigns points as shown in Table 5. Thus, an individual’s PROPKD score can range from 0 to 9 points. Three risk categories of progression to ESRD were subsequently defined: low (0-3 points), intermediate (4-6 points), and high (7-9 points). The predicted median age of onset for ESRD and predicted disease progression for these 3 risk categories are listed in Table 6. Of note, the PROPKD scoring system cannot be applied to patients with no history of urological events or hypertension and has not been widely validated in independent cohorts.
Table 5. The PROPKD Scoring System.
| Factor | Points |
|---|---|
| Male | 1 |
| Hypertension before age 35 years | 2 |
| First urological event before age 35 years | 2 |
| PKD2 mutation | 0 |
| Nontruncating PKD1 mutation | 2 |
| Truncating PKD1 mutation | 4 |
Table 6. Predicted Median Age of Onset of ESRD and Predicted Disease Progression by PROPKD Risk Category.
| PROPKD risk category for progression to ESRD | |||
|---|---|---|---|
| Low risk (0-3 points) | Intermediate risk (4-6 points) | High risk (7-9 points) | |
| Predicted median age of onset for ESRD,y | 70.6 | 56.9 | 49.0 |
| Predicted disease progression | Excludes progression to ESRD before 60 years of age (negative predictive value of 81.4%) | Prognosis is unclear | Rapid progression to ESRD before 60 years of age (positive predictive value of 90.9%) |
| PROPKD risk category for progression to ESRD | |
|---|---|
| Low risk (0-3 points) | |
| Predicted median age of onset for ESRD,y | 70.6 |
| Predicted disease progression | Excludes progression to ESRD before 60 years of age (negative predictive value of 81.4%) |
| Intermediate risk (4-6 points) | |
|---|---|
| Predicted median age of onset for ESRD,y | 56.9 |
| Predicted disease progression | Prognosis is unclear |
| High risk (7-9 points) | |
|---|---|
| Predicted median age of onset for ESRD,y | 49.0 |
| Predicted disease progression | Rapid progression to ESRD before 60 years of age (positive predictive value of 90.9%) |