In a recent NDT paper, the ERA-EDTA Working Groups of Inherited Kidney Disorders and European Renal Best Practice provides guidance for making the decision as to which ADPKD patients to treat with tolvaptan, and presents a hierarchical decision algorithm according to risk-factor assessments in a descending order of reliability.
The key recommendations on tolvaptan use the group makes are:
- We suggest that tolvaptan can be prescribed to adult ADPKD patients aged <50 years with CKD stages 1–3a (eGFR >45 mL/min/1.73 m2) who have demonstrated or who are likely to have rapidly progressing disease. CKD stage must be interpreted in conjunction with age.
- We recommend not starting tolvaptan in patients aged 30–40 years with CKD stage 1.
- We recommend not starting tolvaptan in patients aged 40–50 years with CKD stages 1 or 2 (eGFR >60 mL/min/1.732).
How to define rapid progression:
- A confirmed annual eGFR decline ≥5 mL/min/1.73 m2 in 1 year, and/or ≥2.5 mL/min/1.73 m2 per year over a period of 5 years
- A TKV increase of >5% per year by repeated measurements (preferably three or more, each at least 6 months apart and by MRI).
- Start tolvaptan treatment with a dose of 45 mg in the morning and 15 mg in the evening.
- Uptitrate the dose of tolvaptan to 60/30 and 90/30 mg when tolerated.
- Tolvaptan should be discontinued when patients approach ESRD.
Markers used to assess prognosis in ADPKD
ADPKD Treatment Decision Tree
Click here to access the Mayo Imaging classification tool for ADPKD
The complete guidelines can be accessed at the NDT website.