Jynarque (tolvaptan)

POLICY NUMBER

L.5.01.534

Jynarque (tolvaptan)

Please perform a formulary drug search on your patient’s Member ID to ensure the prescription drug is covered under their benefit plan. The medication(s) in this medical policy may not be covered under a specific Member’s benefit plan.

DESCRIPTION

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of chronic kidney disease and occurs in approximately 1 in 1000 live births. Mutations in two genes have been identified to be the major cause of ADPKD. Mutations in the PKD1 gene (located on chromosome 16) account for about 78% of cases, while mutations in the PKD2 gene (located on chromosome 4) account for about 14% of cases. ADPKD is a systemic disorder characterized by continuous cyst development and growth within the kidneys and other organs, leading to numerous clinical manifestations.

Jynarque (tolvaptan) is a selective vasopressin V2-receptor antagonist indicated to slow kidney function decline in adults at risk of rapidly progressing autosomal dominant polycystic kidney disease (ADPKD).

POLICY

Prior authorization is required.

The use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. 

Initial Criteria

Jynarque (tolvaptan) may be considered medically necessary when ALL of the following are met:

1. The individual is 18 years of age or older;

2. The individual has a documented diagnosis of autosomal dominant polycystic kidney disease (ADPKD) and BOTH of the following:

   1. The individual does NOT have stage 5 chronic kidney disease (CKD); AND

   2. The individual is NOT on dialysis;

3. The individual will NOT be using the requested agent in combination with another tolvaptan agent;

4. The prescriber is a specialist in the area of the individual's diagnosis (e.g., nephrologist) or has consulted with a specialist in the area of the individual's diagnosis;

5. The individual does NOT have any FDA labeled contraindication(s) to the requested medication; AND

6. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: 12 months

Renewal Criteria

Jynarque (tolvaptan) may be approved for RENEWAL when ALL of the following are met:

1. The individual has been previously approved for this medication through the BCBSMS PA process;

2. The individual has documented clinical improvement (i.e. slowed kidney function decline, decreased kidney pain, etc.);

3. The individual will NOT be using the requested agent in combination with another tolvaptan agent;

4. The prescriber is a specialist in the area of the individual's diagnosis (e.g., nephrologist) or has consulted with a specialist in the area of the individual's diagnosis;

5. The individual does NOT have any FDA labeled contraindication(s) to the requested medication; AND

6. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: 12 months

POLICY EXCEPTIONS

State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

B. appropriate with regard to standards of good medical practice; and

C. not solely for the convenience of the Member, his or her Provider; and

D. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

POLICY HISTORY

10/31/2019: New policy added. Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee.

02/01/2023: Policy Exceptions updated to add the following: State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

04/02/2024: Policy description updated regarding autosomal dominant polycystic kidney disease. Policy section updated to revise the initial and renewal criteria for Jynarque (tolvaptan). Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and "Medically Necessary" to "medical necessity." Sources updated.

06/03/2025: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy description updated regarding autosomal dominant polycystic kidney disease. Removed quantity limit table. Policy statement revised to state that the use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. Medically necessary criteria revised regarding age requirement. Renewal criteria updated regarding clinical improvement. Sources updated.

SOURCE(S)

1. Devuyst, Olivier et al. KDIGO 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD). Kidney International, Volume 107, Issue 2, S1 - S239.

2. Jynarque prescribing information. Otsuka America Pharmaceutical, Inc. January 2025. Last accessed March 2025.

3. Torres VE, Bennett WM. Autosomal dominant polycystic kidney disease (ADPKD) in adults: Epidemiology, clinical presentation, and diagnosis. In: UpToDate, Connor RF (Ed), Wolters Kluwer. https://www.uptodate.com/contents/autosomal-dominant-polycystic-kidney-disease-adpkd-in-adults-epidemiology-clinical-presentation-and-diagnosis (Accessed on March 25, 2025)

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.

Medically Necessary Codes

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