CurrentCignaPolicy N/A

Adrenal Hyperplasia - Crenessity Prior Authorization Policy

Defines prior authorization requirements and medical necessity criteria for coverage of Crenessity (crinecerfont) for treatment of classic congenital adrenal hyperplasia (CAH) for Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyAdrenal Hyperplasia - Crenessity Prior Authorization Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateNov 19, 2025

Key ActionObtain prior authorization with specialist prescribing or consultation and supply documentation confirming age, diagnosis, glucocorticoid use, and specialist involvement.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥4minimum age for therapy

6 moinitial approval duration

1 yrrenewal approval

coveredFDA-approved indication

Recommended Authorization Criteria

FDA-Approved Indication

Covered when ONE of the following is met

FDA-Approved Indication: ONE of the following: (A) Initial Therapy OR (B) Currently Receiving Crenessity

A) Initial Therapy: Approve for 6 months if ALL of i, ii, and iii are met6 months

A.i: Patient is ≥ 4 years of age≥ 4 years

A.ii:

Policy Summary

PayerCigna

PolicyAdrenal Hyperplasia - Crenessity Prior Authorization Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateNov 19, 2025

Key ActionObtain prior authorization with specialist prescribing or consultation and supply documentation confirming age, diagnosis, glucocorticoid use, and specialist involvement.

SourceLink

On This Page

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Clinical Trial & Coding Notes

Stable glucocorticoid dose used in trials — > 12 mg/m2/day

Stable glucocorticoid dose used in pivotal trials> 12 mg/m2/day (hydrocortisone dose equivalents)

Context in trialsRequired for enrollment in both pivotal studies evaluating Crenessity (pediatric and adult populations)

Outcome related to thresholdWith baseline glucocorticoid ≥ 12 mg/m2/day, Crenessity significantly decreased serum androstenedione versus placebo at Week 4

Effect on glucocorticoid dosingTotal glucocorticoid dose while androstenedione controlled was significantly reduced in the Crenessity group at Week 24

Prior Authorization, Documentation & Prescribing Requirements

Prior Authorization

Prior Authorization Required

Prior Authorization Recommended. Initial approvals require that Crenessity be prescribed by or in consultation with a physician who specializes in the condition being treated (examples: endocrinologist, urologist, or a physician specializing in adrenal hyperplasia). Approvals are provided for the duration noted in the policy and, when expressed in months, 1 month = 30 days.

Initial approval: specialist prescriber or documented specialist consultation required
Approval durations follow policy-specific timeframes (e.g., 6 months for initial therapy, 1 year for continuation)

Documentation Required

Adjunctive Therapy Requirement

Crenessity must be used as adjunctive therapy in combination with a systemic glucocorticoid (examples: hydrocortisone, prednisone, prednisolone, or dexamethasone). Requests that do not document concomitant systemic glucocorticoid therapy do not meet the adjunctive therapy requirement.

Medication must be prescribed to be taken in combination with a systemic glucocorticoid
Examples of acceptable glucocorticoids: hydrocortisone, prednisone, prednisolone, dexamethasone

Documentation Required

Required Clinical Documentation

Required clinical documentation includes patient age and diagnosis confirmation for 21‑hydroxylase deficiency CAH (one of: elevated 17‑hydroxyprogesterone level; confirmed CYP21A2 genotype; positive newborn screening with confirmatory second‑tier testing; or diagnostic results after cosyntropin stimulation). For continuation requests, documentation that the patient is deriving clinical benefit (for example: reduced androstenedione or 17‑hydroxyprogesterone levels, reduced glucocorticoid dose from baseline, or improved/stable CAH signs/symptoms) should be provided.

Document patient age (initial therapy requires age ≥ 4 years)
Documentation of CAH diagnosis by one of the specified laboratory/genetic/testing methods
For continuation: evidence of clinical benefit or stability

Denial Risk

Non‑Covered Uses / Denial Risk

Non‑covered uses: Requests that do not meet the authorization criteria or are for any other indications not listed in the Recommended Authorization Criteria are considered not medically necessary.

Coverage not recommended for circumstances not listed in the Recommended Authorization Criteria
Criteria will be updated as new published data become available

Clinical Background

Classic congenital adrenal hyperplasia (CAH) due to 21‑hydroxylase deficiency is the most common cause of primary adrenal insufficiency in children and often presents with impaired cortisol synthesis and androgen excess. It is frequently detected by newborn screening (elevated 17‑hydroxyprogesterone) and may be confirmed by CYP21A2 genotype or cosyntropin stimulation testing. Management requires glucocorticoid replacement—often at supraphysiologic doses—and adjunctive therapies such as Crenessity are indicated to help control androgen excess in appropriately selected patients.

Definitions

Classic congenital adrenal hyperplasia (CAH)

DefinitionClassic congenital adrenal hyperplasia (CAH) is a genetic disorder, most commonly due to 21-hydroxylase (CYP21A2) deficiency, leading to impaired cortisol synthesis

Common cause21-hydroxylase deficiency (CYP21A2) is the most common cause of classic CAH

Typical diagnostic markersElevated 17-hydroxyprogesterone on newborn screening; confirmatory testing may include cosyntropin stimulation or CYP21A2 genetic testing

Clinical significanceClassic CAH is the most common cause of primary adrenal insufficiency in children and may present on newborn screening or with atypical genitalia in females

Management noteManagement requires adequate glucocorticoid replacement, often at supraphysiological hydrocortisone doses