CurrentCignaPolicy N/A

Zokinvy (lonafarnib) prior authorization

Defines prior authorization requirements, coverage criteria, and exclusions for Zokinvy (lonafarnib) for Cigna-administered health benefit plans, including prescriber specialty and diagnostic requirements for approval.

Policy Summary

PayerCigna

PolicyZokinvy (lonafarnib) prior authorization

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionObtain prior authorization and document confirmed pathogenic LMNA variant consistent with HGPS; approvals are for 1 year when criteria are met.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥ 12 monthsminimum patient age for coverage

≥ 0.39 m2minimum body surface area for coverage

1 yearauthorization duration

Not medically necessaryprogeroid laminopathies stance

02/04/2026policy review

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

FDA-Approved Indication — Hutchinson-Gilford Progeria Syndrome

Approve for 1 year if the patient meets ALL of the following (A, B, C, and D):

HGPS approval criteria: A) Patient is ≥ 12 months of age;

HGPS approval criteria: B) Patient has a body surface area of ≥ 0.39 m2;≥ 0.39 m2

HGPS approval criteria: C) Genetic testing demonstrates a confirmed pathogenic variant in the LMNA gene consistent with Hutchinson-Gilford Progeria Syndrome;

Refer to Appendix for listing of genetic variants consistent with HGPS.

HGPS approval criteria: D) The medication is prescribed by or in consultation with a geneticist or pediatric cardiologist.

Zokinvy (lonafarnib) is not medically necessary for the treatment of progeroid laminopathies and other progeroid syndromes. The efficacy of Zokinvy has not been established for genetic disorders other than Hutchinson-Gilford Progeria Syndrome (HGPS), and available evidence does not demonstrate a treatment effect in these populations. In addition, Zokinvy is not indicated for processing-proficient progeroid laminopathies and would not be expected to be effective based on its mechanism of action.

Use of Zokinvy for progeroid laminopathies and other progeroid syndromes is considered not medically necessary. FDA labeling and available clinical data do not support benefit of lonafarnib outside of HGPS, and there are no clinical data demonstrating a treatment effect in these related disorders.

Initial Therapy

Initial Therapy — Initial authorization criteria for FDA-approved use

Initial authorization criteria for FDA-approved use

Initial authorization for HGPS: Patient ≥ 12 months old; BSA ≥ 0.39 m2; confirmed pathogenic LMNA variant consistent with HGPS; prescribed by or in consultation with a geneticist or pediatric cardiologist.

Authorize for 1 year.

Provider Actions and Authorization Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for coverage of Zokinvy (lonafarnib). Approvals are provided for 1 year when criteria for the FDA‑approved indication are met. Because of the specialized skills required for evaluation, diagnosis, and monitoring of patients treated with Zokinvy, the medication must be prescribed by or in consultation with a geneticist or pediatric cardiologist.

Prior authorization required — approve for 1 year when FDA‑approved criteria are met.
Medication must be prescribed by or in consultation with a geneticist or pediatric cardiologist.
Denial risk if requests do not meet prior authorization criteria (including absence of required diagnostic confirmation, age, body surface area, or other criteria).

Documentation Required

Required Documentation

Genetic testing demonstrating a confirmed pathogenic variant in the LMNA gene consistent with Hutchinson‑Gilford Progeria Syndrome (HGPS) is required. Refer to the Appendix for specific genetic variants associated with HGPS.

Required documentation: genetic test results confirming a pathogenic LMNA variant (see Appendix for listed variants).

Note

Provider Action Items

Highlight provider and billing actions: ensure prior authorization is obtained, include genetic testing documentation and patient age/body surface area information with the request, and document specialist involvement (geneticist or pediatric cardiologist). Requests missing required diagnostic confirmation, age, body surface area, or specialist prescribing/consult note may be denied.

Include patient age (must be ≥ 12 months) and body surface area (must be ≥ 0.39 m2) with the prior authorization request.
Attach genetic testing confirming a pathogenic LMNA variant.
Document that the medication is prescribed by or in consultation with a geneticist or pediatric cardiologist.
Anticipate denial if criteria are not met or documentation is incomplete.

Definitions

HGPS — definition and genetic variants

DefinitionHutchinson-Gilford Progeria Syndrome (HGPS) — an ultra-rare, fatal, autosomal dominant genetic disorder caused by heterozygous LMNA mutations that produce progerin, leading to premature aging and rapidly progressive atherosclerosis.

Typical genetic variantClassic HGPS is most commonly caused by the LMNA c.1824C>T (p.G608G) single point variant, which accounts for ~90% of cases.

Other LMNA variantsNon-classic HGPS (~10% of cases) involves variants at the exon 11 splice junction or intron 11 that activate a cryptic splice site, also producing progerin (see Appendix for full listing).

Clinical consequenceProgerin accumulation causes nuclear membrane stiffening and disorganized nuclear architecture, resulting in rapidly progressive atherosclerosis and early mortality (average 14.6 years).

EpidemiologyEstimated incidence ~1:4,000,000 live births; prevalence ~1:20,000,000; 17 patients with HGPS identified in the U.S. as of Dec 31, 2025.

Progeroid laminopathies — definition and relation to HGPS

DefinitionProgeroid laminopathies — a group of lamin A processing–related disorders with phenotypic overlap to HGPS, arising from diverse LMNA variants or pathogenic ZMPSTE24 variants.

Relation to HGPSSome progeroid laminopathies present similarly to HGPS (premature aging features) but represent a broader set of laminopathies with variable phenotypes and less direct evidence for Zokinvy benefit.

ZMPSTE24-related disordersPathogenic variants in ZMPSTE24 can cause excess prelamin A protein and a related progeroid phenotype; these are included among processing-deficient progeroid laminopathies referenced in product labeling.

Evidence noteMortality and treatment efficacy data for Zokinvy in progeroid laminopathies are limited; pivotal trials primarily included patients with HGPS, and outcomes for progeroid laminopathies are not established.

Background

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare, fatal, autosomal dominant disorder caused primarily by pathogenic variants in the LMNA gene that result in accumulation of the mutant protein progerin. Progerin accumulation leads to widespread premature aging changes, notably accelerated atherosclerosis, and substantially reduced life expectancy. Zokinvy is a protein farnesyltransferase inhibitor indicated to reduce mortality in patients with HGPS; however, evidence for benefit in other progeroid conditions is limited or absent.