CurrentCignaPolicy IP0587

Pozelimab-bbfg (Veopoz) for CD55-deficient protein-losing enteropathy (CHAPLE disease)

Defines medical necessity, dosing, authorization durations, reauthorization requirements, and coding for pozelimab-bbfg (Veopoz) for treatment of CHAPLE disease for Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyPozelimab-bbfg (Veopoz) for CD55-deficient protein-losing enteropathy (CHAPLE disease)

Policy CodePolicy IP0587

Change TypeCoding and clinical criteria revisions

Effective DateNov 15, 2024

Next Review Date

Key ActionObtain prior authorization (initial 3 months) and document biallelic CD55 loss-of-function mutations, serum albumin ≤ 3.2 g/dL, evidence of active disease within the last 6 months, updated vaccinations per ACIP, and specialist involvement to support coverage.

SourceLink

POLICY UPDATE CHANGES

Removed the statement 'Does not have active meningococcal infection'.

Updated coding: Added J9376 and removed C9399, J3490, J3590.

≥1Minimum patient age for coverage

3Authorization duration - initial

12Authorization duration - renewal

30 mg/kgInitial dosing (loading)

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Medically Necessary Criteria

Medically Necessary — Initial Therapy for CHAPLE Disease

Pozelimab-bbfg (Veopoz) is considered medically necessary when ALL of the following are met:

ALL of the following

Patient is age 1 year or older
Minimum age requirement for coverage
Diagnosis of CHAPLE disease with biallelic CD55 loss-of-function mutations as documented by genetic testing
Laboratory and clinical activity
- Serum albumin level less than or equal to 3.2 g/dL<= 3.2 g/dL
- Has active disease and is experiencing one or more signs or symptoms within the last 6 months (for example, abdominal pain, diarrhea, vomiting, peripheral edema, or facial edema)
Vaccination requirements
- Has received or is in compliance with updated meningococcal vaccinations according to the most current Advisory Committee on Immunization Practices recommendations
- Has received or is in compliance with updated vaccinations for the prevention of Streptococcus pneumoniae and Haemophilus influenzae type b infections according to the most current Advisory Committee on Immunization Practices guidelines
Medication is prescribed by, or in consultation with, a physician with expertise in managing CHAPLE disease
Specialist involvement required

Reauthorization / Continuation Criteria

Continuation is medically necessary when ALL of the following are met:

ALL of the following

Original medical necessity criteria for CHAPLE disease continue to be met
- Examples include increased serum albumin levels or maintenance of serum albumin within normal range
- Reduction in albumin transfusions
- Increases in or maintenance of protein and/or immunoglobulin levels
- Improvement in clinical outcomes after receipt of therapy (e.g., reduced frequency of hospitalizations, increased growth percentiles, reduced use of corticosteroids)

Not Medically Necessary / Experimental or Unproven Uses

Any use other than the indication and criteria listed is considered experimental, investigational, or unproven, including:

ANY of the following

Concomitant use with other complement inhibitors (prohibited in pivotal study)

Relevant Billing Codes

Covered HCPCSHCPCSCovered

J9376

Injection, pozelimab-bbfg, 1 mg

Authorization, Documentation, and Billing Rules

Prior Authorization

Prior authorization required

Authorization is required for pozelimab‑bbfg (Veopoz). Initial approval is for 3 months; reauthorization requires documentation of clinical benefit and, if approved, will be granted for 12 months.

Affected code: J9376
Initial: 3 months
Reauthorization: 12 months

Documentation Required

Genetic testing documentation

Provider must document biallelic CD55 loss-of-function mutations by genetic testing to support the diagnosis of CHAPLE disease.

Documentation Required

Clinical Thresholds, Authorization Durations, and Dosing Limits

Key Thresholds and Dosing

Serum albumin<= 3.2 g/dL

Minimum age>= 1 year

Initial authorization duration3 months

Reauthorization duration12 months

Loading dose30 mg/kg IV on Day 1

Age ≥ 1 yearMinimum patient age for coverage

3 monthsAuthorization duration - initial

Clinical Background

Veopoz (pozelimab‑bbfg) is a complement inhibitor indicated for treatment of CD55‑deficient protein‑losing enteropathy (CHAPLE disease) in patients aged 1 year and older. CHAPLE disease (Complement Hyperactivation, Angiopathic thrombosis, and Protein‑Losing Enteropathy) is an ultra‑rare, inherited disorder caused by biallelic CD55 loss‑of‑function mutationscomplement system hyperactivation, resulting in protein‑losing enteropathy and symptoms such as abdominal pain, diarrhea, vomiting, impaired growth, weight loss, and edema. Severe thrombotic vascular occlusions and increased morbidity and mortality can occur in affected patients.

Veopoz has a Boxed Warning for serious meningococcal infections. Life‑threatening and fatal meningococcal infections have been reported with complement inhibitors. Complete or update meningococcal vaccination at least 2 weeks before the first dose of Veopoz is recommended per the most current Advisory Committee on Immunization Practices (ACIP) guidance, unless the risks of delaying therapy outweigh the risks of infection. Patients receiving Veopoz are also at increased risk for invasive disease from Streptococcus pneumoniae and Haemophilus influenzae type b, and related vaccinations should be administered according to ACIP recommendations.

Defined Terms

Definitions

CHAPLE diseaseCD55-Deficient Protein-Losing Enteropathy (Complement Hyperactivation, Angiopathic thrombosis, and Protein-Losing Enteropathy) caused by biallelic CD55 loss-of-function mutations.

Beneficial responseClinical or laboratory improvement such as increased or maintained serum albumin, reduced albumin transfusions, improved protein/immunoglobulin levels, fewer hospitalizations, improved growth percentiles, or reduced corticosteroid use.

Policy Changes

2024-11-15Revised — Clinical criteriaLatest

Removed the explicit requirement stating 'Does not have active meningococcal infection' from the CHAPLE disease medical necessity criteria.

2024-11-15Revised — Coding

Consolidated HCPCS coding: added J9376 (Injection, pozelimab-bbfg, 1 mg) and removed codes C9399, J3490, and J3590.