Subject: Jynarque 2572-A SGM P2022

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 method⁷
      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 method⁷
      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 method⁷
      4.   In members with no family history (no first degree relative with disease): positive genetic test for ADPKD
            (mutation in PKD1 or PKD2 gene)³
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 disease⁵
C.   The member’s estimated glomerular filtration rate (eGFR) is greater than or equal to 25 mL/min/1.73m².^1,6 

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. Gansevoort RT, Arici M, Benzing T, et al. Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice. Nephrol Dial Transplant. 2016;31(3):337-348.
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 17, 2022