Guidelines: Use of Tolvaptan in Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Tolvaptan, ADPKDRecently, the European Drug Administration approved the use of vasopressin V2 receptor antagonist - Tolvaptan to delay the development of autosomal dominant Polycystic Kidney Disease (ADPKD) cysts and renal insufficiency in adults with Chronic Kidney Disease (CKD).

Professor Ron T. Gansevoort of Nephrology Department, University of Groningen Medical Center, Netherlands, on behalf of the ERA-EDTA Working Group on Hereditary Kidney Diseases and European Nephropathy Best Practices, published a guideline on the recommendation of Tolvaptan for patients with ADPKD in the March 2016 issue of the Journal of Nephrology Dialysis and Transplantation. The main points are summarized as follows:

Recommendation 1

1. It is recommended that tolvaptan be used in adult ADPKD patients with CKD stage 1-3a (estimated glomerular filtration rate EGFR > 45ml/min/1.73m2) and age <50 years who have rapid progression of renal function. However, CKD staging must be interpreted in conjunction with age.

2. It is not recommended that tolvaptan be used in ADPKD patients with stage 1 CKD (eGFR>90 ml/min/1.73m2) aged 30-40 years.

3. It is not recommended that tolvaptan be used in ADPKD patients with stage 1-2 CKD (eGFR > 60 ml/min/1.73 m2) aged 40-50 years.

Recommendation 2

Rapid progression of renal function is defined as a decrease of eGFR ≥ 5 ml/min/1.73 m2) in one year and ≥ 2.5 ml/min/1.73 m2 in five years.

Recommendation 3

Repeated total kidney volume (TKV) increases by more than 5% annually are defined as rapid progression of disease. It is preferable to use magnetic resonance imaging (MRI) for at least three measurements at a time interval of at least six months.

Recommendation 4

1. It is recommended that Mayo's ADPKD classification be used to distinguish between "typical form" and "atypical form", and TKV of "typical form" patients should be corrected according to age and height, and five types (1A-1E) of patients should be defined according to prognosis.

2. It is believed that in patients with Mayo 1C~1E (corresponding predicted annual eGFR reduction ≥ 2.5 ml/min/1.73 m2) ADPKD, the disease may progress rapidly.

3. It is believed that in ADPKD patients described as "atypical form" in Mayo typing, disease progression is unlikely to be rapid.

4. It is believed that the disease may progress rapidly in patients aged < 45, and kidney cyst size > 16.5 cm.

Recommendation 5

It is believed that in patients with PKD1 mutation and early onset of clinical symptoms (consistent with the PRO-PKD score > 6), the disease may progress rapidly.

Recommendation 6

It is believed that in patients with a family history of end-stage renal disease (ESRD) before the age of 58, rapid progression of the disease is reassessessed every three to five years.

Recommendation 7

It is recommended that a hierarchical decision-making algorithm be used to assess whether or not ADPKD patients are or may be rapidly progressing patients and to determine whether they are eligible for Tolvaptan treatment.

Recommendation 8

1. It is recommended to discuss adverse drug reactions and lifestyle impacts with patients when considering starting Tolvaptan treatment.

2. It is recommend that hepatotoxicity and other considerations listed below be taken into account when considering the initiation of Tolvaptan therapy.

3. It is recommended Tolvaptan prescription and safety monitoring under the supervision of experienced doctors who treat ADPKD.

Recommendation 9

1. It is recommended that the initial dosage of Tolvaptan be 45 mg in the morning and 15 mg in the evening.

2. It is recommended that if tolerated, Tolvaptan should be titrated gradually to 60 mg/evening 30 mg in the morning and 90 mg/evening 30 mg in the morning.

3. It is recommended stopping Tolvaptan treatment when patients approach ESRD.

Now you know the use of Tolvaptan in ADPKD. For more information on its treatment, please leave a message below or contact online doctor.


***Please seek professional medical advise for the diagnosis or treatment of any ailment, disease or medical condition. This article is not intended to be a substitute for the advice of a licensed medical professional.***

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