CurrentCignaPolicy N/A

Adrenal Hyperplasia - Crenessity Prior Authorization Policy

This policy governs prior authorization requirements for Crenessity (crinecerfont capsules and oral solution) when used to treat classic congenital adrenal hyperplasia (CAH) in covered Cigna health benefit plans.

Policy Summary

PayerCigna

PolicyAdrenal Hyperplasia - Crenessity Prior Authorization Policy

Policy CodePolicy N/A

Change TypeNo material change

Effective DateN/A

Next Review DateDec 18, 2024

Key ActionObtain prior authorization with documentation confirming diagnosis of 21‑hydroxylase deficiency CAH and that Crenessity will be used with a systemic glucocorticoid.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥4minimum age

6 moinitial approval

1 yrrenewal approval

>12 mg/m2trial steroid dose

Not coveredother uses

Coverage Criteria for Crenessity (crinecerfont)

Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met

Initial Therapy for Classic CAH: Patient is ≥ 4 years of age

Concomitant glucocorticoid therapy: Medication will be taken in combination with a systemic glucocorticoid (examples: hydrocortisone, prednisone, prednisolone, or dexamethasone)

Confirmed diagnosis of 21-hydroxylase deficiency CAH: Diagnosis is confirmed by ONE of: (1) elevated 17-hydroxyprogesterone level; OR (2) confirmed CYP21A2 genotype; OR (3) positive newborn screening with confirmatory second-tier testing; OR (4) diagnostic results after cosyntropin stimulation

Specialist prescribing:

Policy Summary

PayerCigna

PolicyAdrenal Hyperplasia - Crenessity Prior Authorization Policy

Policy CodePolicy N/A

Change TypeNo material change

Effective DateN/A

Next Review DateDec 18, 2024

Key ActionObtain prior authorization with documentation confirming diagnosis of 21‑hydroxylase deficiency CAH and that Crenessity will be used with a systemic glucocorticoid.

SourceLink

On This Page

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Medication is prescribed by or in consultation with an endocrinologist, urologist, or physician who specializes in the treatment of adrenal hyperplasia

Initial approval duration: 6 months.

Continuation Therapy — Covered when ANY of the following clinician-documented benefits are present (per prescriber)

Covered when ANY of the following clinician-documented benefits are present (per prescriber)

Continuation Therapy for Current Users

Examples of clinician-documented benefits: Reduced androstenedione levels
Decreased 17-hydroxyprogesterone levels
Reduction in glucocorticoid dose from baseline (prior to Crenessity therapy)
Improved or stabilized clinical signs/symptoms (e.g., decrease in BMI z-score, improved insulin resistance, reduction of hirsutism, or improved androstenedione-to-testosterone ratio)

Crenessity (crinecerfont) is considered experimental, investigational, or unproven when used for any indication other than classic congenital adrenal hyperplasia (CAH). Requests for coverage for non‑CAH indications do not meet the criteria of this policy and may be denied; the policy will be updated if new evidence becomes available.

Use of Crenessity for indications other than classic congenital adrenal hyperplasia is not supported by current evidence and is not covered under this policy. Providers seeking coverage for off‑label or alternative indications should note that such uses are considered investigational and are not eligible for authorization pending publication of supportive data.

Drug Coding and Trial Dose Information

Covered DrugNDCCovered

NDC (not listed)

Crenessity (crinecerfont) capsules and oral solution

Stable glucocorticoid dose in trials (hydrocortisone equivalents)

Trial glucocorticoid requirement> 12 mg/m2/day (hydrocortisone equivalents)

Context of thresholdBoth pivotal studies required a stable glucocorticoid dose > 12 mg/m2/day prior to enrollment

Dose unitsHydrocortisone dose equivalents (mg/m2/day)

Associated efficacy outcomeStudies showed significant decrease in serum androstenedione versus placebo with this background glucocorticoid dose

Provider Requirements, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for Crenessity. Approvals are time-limited as specified in the coverage criteria (initial therapy approvals for 6 months; continuation/currently receiving approvals for 1 year). Initial approval requires the medication to be prescribed by or in consultation with a physician who specializes in the condition being treated.

Crenessity requires prior authorization
Approval durations: Initial therapy = 6 months; Continuing therapy = 1 year

Step Therapy

No step therapy requirements are specified for Crenessity in this policy.

Documentation Required

Required documentation for Prior Authorization

Documentation must be provided showing the patient meets the indication-specific criteria for classic congenital adrenal hyperplasia (CAH) when requesting prior authorization.

Patient age ≥ 4 years for initial therapy
Medication will be taken in combination with a systemic glucocorticoid (e.g., hydrocortisone, prednisone, prednisolone, dexamethasone)
Diagnosis of 21-hydroxylase deficiency CAH confirmed by ONE of the following: (1) elevated 17-hydroxyprogesterone level; (2) confirmed CYP21A2 genotype; (3) positive newborn screening with confirmatory second-tier testing; (4) diagnostic results after cosyntropin stimulation
For initial therapy: medication prescribed by or in consultation with an endocrinologist, urologist, or physician who specializes in adrenal hyperplasia
For continuation therapy: documentation from the prescriber that the patient is continuing to derive benefit (examples: reduced androstenedione or 17‑hydroxyprogesterone levels, reduction in glucocorticoid dose from baseline, improved/stabilized clinical signs/symptoms such as decreased BMI SDS, improved insulin resistance, reduction of hirsutism, or improved androstenedione-to-testosterone ratio)

Denial Risk

Non‑approved indications

Use of Crenessity for any indication other than classic CAH is considered experimental, investigational, or unproven and is not covered.

Key Definitions

Classic CAH — key points

DefinitionClassic congenital adrenal hyperplasia (CAH) due to 21‑hydroxylase deficiency

Indication for CrenessityCrenessity is indicated as adjunctive treatment to glucocorticoid replacement to control androgens in patients with classic CAH aged ≥ 4 years

Typical presentation and diagnosisOften detected on newborn screening for elevated 17‑hydroxyprogesterone; diagnosis may be confirmed by cosyntropin stimulation testing or CYP21A2 genotyping

Epidemiology/clinical noteMost common cause of primary adrenal insufficiency in children; prevalence ~1 in 10,000–15,000 live births

Background

Classic congenital adrenal hyperplasia (CAH) due to 21‑hydroxylase deficiency is the most common cause of primary adrenal insufficiency in children and is commonly detected through newborn screening for elevated 17‑hydroxyprogesterone. Management of classic CAH typically requires supraphysiological glucocorticoid replacement to control androgen excess. Crenessity is indicated as an adjunct to a systemic glucocorticoid for patients with classic CAH aged ≥4 years; information on disease prevalence, detection, and treatment rationale is summarized here to support appropriate use.

This policy limits coverage of Crenessity to the FDA‑approved population with classic CAH. Other uses are considered experimental or unproven and are not covered; the policy’s "Conditions Not Covered" section will be updated if new published data become available.