Subject: Juxtapid 2071-A SGM P2022
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
Juxtapid is indicated as an adjunct to a low-fat diet and other lipid-
lowering treatments, including LDL apheresis where available, to reduce
low-density lipoprotein cholesterol (LDL-C), total cholesterol (TC),
apolipoprotein B (apo B), and non-high-density lipoprotein cholesterol
(non-HDL-C) in patients with homozygous familial hypercholesterolemia
(HoFH).
Limitations of Use:
• The safety and effectiveness of Juxtapid have not been established
in patients with hypercholesterolemia who do not have HoFH,
including those with heterozygous familial hypercholesterolemia
(HeFH).
• The effect of Juxtapid on cardiovascular morbidity and mortality
has not been determined.
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. Current LDL-C level for both initial requests and continuation requests.
The level must be dated within the six months preceding the
authorization request.
B. Genetic testing or medical records confirming the diagnosis of HoFH.
C. Medical records confirming the member is currently on lipid lowering
therapy for both initial requests and continuation requests
III. CRITERIA FOR INITIAL APPROVAL
Homozygous familial hypercholesterolemia (HoFH)
Authorization of 6 months may be granted for treatment of homozygous
familial hypercholesterolemia when all of the following criteria are met:
A. Member has a documented diagnosis of homozygous familial
hypercholesterolemia confirmed by any of the following criteria:
1. Mutations in two alleles at the LDLR, APOB, PCSK9 or LDLRAP1
gene locus
2. An untreated LDL-C of greater than 500 mg/dL or treated LDL-C
greater than or equal to 300 mg/dL and either of the following:
a. Presence of cutaneous or tendinous xanthomas before the age
of 10 years
b. An untreated LDL-C level of greater than or equal to 190 mg/dL in both parents
B. Prior to initiation of treatment with the requested medication, both
of the following criteria are/were met:
1. Member is/was receiving a combination lipid-lowering regimen
consisting of a high-intensity statin, ezetimibe, and PCSK9 directed
therapy unless the member has known LDL-receptor negative
mutations in both alleles.
2. Member is/was experiencing an inadequate response to such a
combination regimen, as demonstrated by a treated LDL-C of
greater than or equal to 100 mg/dL (or greater than or equal to 70
mg/dL with clinical atherosclerotic cardiovascular disease
[ASCVD]), unless the member has known LDL-receptor negative
mutations in both alleles.
C. Member will continue to receive concomitant lipid-lowering therapy.
IV. CONTINUATION OF THERAPY
Authorization of 12 months may be granted for continued treatment in
members (including new members) who meet all of the following criteria:
A. Member meets all initial authorization criteria
B. Member has achieved or maintained an LDL-C reduction greater than
20% from the levels immediately prior to initiation of treatment with
the requested medication
C. Member is currently receiving concomitant lipid-lowering therapy
Place of Service:
Outpatient
The above policy is based on the following references:
- Juxtapid [package insert]. Cambridge, MA: Aegerion Pharmaceuticals, Inc.; September 2020.
- Nordestgaard BG, Chapman MJ, Humphries SE, et al. Familial hypercholesterolaemia is underdiagnosed and undertreated in the general population: guidance for clinicians to prevent coronary heart disease. Consensus Statement of the European Atherosclerosis Society. Eur Heart J. 2013; 34:3478–3490.
- Cuchel M, Bruckert E, Ginsberg HN, et al. Homozygous familial hypercholesterolaemia: new insights and guidance for clinicians to improve detection and clinical management. A position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society. Eur Heart J. 2014; 35:2146-2157.
- National Institute for Health and Clinical Excellence (2008). Identification and management of familial hypercholesterolaemia. NICE clinical guideline 71.
- Cuchel M, Meagher EA, du Toit Theron H, et al. Efficacy and safety of a microsomal triglyceride transfer protein inhibitor in patients with homozygous familial hypercholesterolaemia: a single-arm, open-label, phase 3 study. Lancet. 2013; 381:40-46.
- Goldberg AC, Hopkins PN, Toth PP, et al. Familial hypercholesterolemia: screening, diagnosis and management of pediatric and adult patients. Clinical guidance from the National Lipid Association Expert Panel on Familial Hypercholesterolemia. J Clin Lipidol. 2011; 5:S1–S8.
- Raal JF, Santos RD. Homozygous familial hypercholesterolemia: current perspectives on diagnosis and treatment. 2012; 223:262-268.
- Bays HE, Jones PH, Orringer CE, et al. National Lipid Annual Summary of Clinical Lipidology 2016. J Clin Lipidol 2016; 10:S1-S43.
- Lloyd-Jones DM, Morris PB, Ballantyne CM, Birtcher KK, Daly DD Jr., DePalma SM, Minissian MB, Orringer CE, Smith SC Jr. 2017 focused update of the 2016 ACC expert consensus decision pathway on the role of non-statin therapies for LDL-cholesterol lowering in the management of atherosclerotic cardiovascular disease risk: a report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents. J Am Coll Cardiol 2017;70:1785–822.
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.
May 02, 2022
