Subject: Jynarque 2572-A SGM P2022a

Drug

JYNARQUE  (tolvaptan)

Policy:

      I.  INDICATIONS

          The indications below including FDA-approved indications and
          compendial uses are considered a covered benefit provided that
          all the approval criteria are met and the member has no
          exclusions to the prescribed therapy.

          FDA-Approved Indication
          Jynarque is indicated to slow kidney function decline in adults at
          risk of rapidly progressing autosomal dominant polycystic kidney
          disease (ADPKD).

          All other indications are considered experimental/investigational
          and not medically necessary.

 
    II.  DOCUMENTATION

          Submission of the following information is necessary to initiate
          the prior authorization review:
          A.  Imaging used for diagnosis and confirmation of rapidly
               progressing disease (ultrasonography, magnetic resonance
               imaging [MRI], computed tomography [CT])
          B.  Genetic testing results if applicable

 
  III.  CRITERIA FOR INITIAL APPROVAL

          Authorization of 12 months may be granted for treatment of
          autosomal dominant polycystic kidney disease (ADPKD) when all
          of the following criteria are met:
          A.  The member is 18 years of age or older and with a diagnosis
               of ADPKD as confirmed by any of the following:
               1.  In members aged 18 to less than 40 years with a first
                    degree relative with ADPKD: greater than or equal to 3
                    cysts (unilateral or bilateral) using any radiologic method
               2.  In members aged 40 to less than 60 years with a first
                    degree relative with ADPKD: greater than or equal to 2
                    cysts per kidney using any radiologic method
               3.  In members aged 60 or older with a first degree relative
                    with ADPKD: greater than or equal to 4 cysts per kidney
                    using any radiologic method
               4.  In members with no family history (no first degree
                    relative with disease): positive genetic test for ADPKD
                    (mutation in PKD1 or PKD2 gene)
          B.  The member has or is at risk for rapidly progressing disease
               as confirmed by height-adjusted total kidney volume
               compatible with Mayo class 1C, 1D, or 1E disease
          C.  The member’s estimated glomerular filtration rate (eGFR) is
               greater than or equal to 25 mL/min/1.73m²

 
   IV.  CONTINUATION OF THERAPY

          Authorization of 12 months may be granted for continued
          treatment in members requesting reauthorization for an
          indication listed in Section III when the member has
          demonstrated a beneficial response to Jynarque therapy (e.g.,
          slowed kidney function decline, decreased kidney pain) and the
          member’s estimated glomerular filtration rate (eGFR) is greater
          than or equal to 25 mL/min/1.73m².

Place of Service:

Outpatient

The above policy is based on the following references:

1. Jynarque [package insert]. Rockville, MD: Otsuka America Pharmaceutical, Inc.; October 2020.
2. Torres VE, Devuyst O, Chapman AB, et al; for the REPRISE Trial Investigators. Rationale and design of a clinical trial investigating tolvaptan safety and efficacy in autosomal dominant polycystic kidney disease. Am J Nephrol. 2017;45(3):257-266.
3. Chapman AB, Devuyst O, Eckardt KU, et al. Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int. 2015;88(1):17-27.
4. Srivastava A, Patel N. Autosomal dominant polycystic kidney disease. Am Fam Physician. 2014 Sep 1;90(5):303-307.
5. Müller RU, Messchendorp AL, Birn H, et al. An update on the use of tolvaptan for autosomal dominant polycystic kidney disease: consensus statement on behalf of the ERA Working Group on Inherited Kidney Disorders, the European Rare Kidney Disease Reference Network and Polycystic Kidney Disease International. Nephrol Dial Transplant. 2022;37(5):825-839.
6. Pei Y, Obaji J, Dupuis A, et. Al. Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2009;20:205-212.

Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.

April 11, 2023