CurrentCignaPolicy IP0307

Metabolic Disorders - Xuriden

Policy defines prior authorization and medical necessity criteria for coverage of Xuriden oral granules for treatment of hereditary orotic aciduria (orotic aciduria type 1), including required diagnostic confirmation, prescriber specialty, documentation, duration of approval, and exclusions.

Policy Summary

PayerCigna

PolicyMetabolic Disorders - Xuriden

Policy CodePolicy IP0307

Change TypeSelected and Annual Revisions

Effective Date

Next Review Date

Key ActionObtain prior authorization and ensure Xuriden is prescribed by or in consultation with a metabolic specialist, geneticist, or physician specializing in the condition; provide required documentation to support diagnostic confirmation.

SourceLink

POLICY UPDATE CHANGES

Updated disease name to 'Hereditary Orotic Aciduria (Orotic Aciduria Type 1)' and added requirement for a first-degree family relative with hereditary orotic aciduria.

Policy title updated from 'Uridine triacetate' to 'Metabolic Disorders - Xuriden' and added documentation requirement statement.

1Covered Indication

1 yearTypical Approval Duration

rareDisease Prevalence

Scope: Policy defines prior authorization and medical necessity criteria for coverage of Xuriden (uridine triacetate) oral granules for treatment of hereditary orotic aciduria (orotic aciduria type 1). Subject: Xuriden (uridine triacetate) for hereditary orotic aciduria. Policy number: IP0307. Coverage stance: covered_with_criteria. Status: CURRENT. Last review date: 2025-11-01. Xuriden is covered when diagnostic and prescriber criteria are met; typical approval duration is 1 year. Disease is very rare: estimated prevalence rare (<1:1,000,000).

Key policy facts

PolicyMetabolic Disorders - Xuriden

Policy identifierIP0307

Coverage stanceCovered with criteria

Typical Approval Duration1 year

Disease Prevalence (approximate)rare (<1:1,000,000)

Last review date2025-11-01

Medical Necessity Criteria

Medically Necessary / Initial approval (Xuriden)

Xuriden oral granules are considered medically necessary when the following are met:

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title

nodes

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Not Medically Necessary / Exclusions

Conditions not covered

ALL of the following

Use of Xuriden for any indication other than hereditary orotic aciduria (orotic aciduria type 1) is considered not medically necessary
Receipt of sample product does not satisfy any criteria requirements for coverage

Provider Actions

Prior Authorization

Prior authorization required

Prior Authorization is required for benefit coverage of Xuriden; approval requires the medication to be prescribed by or in consultation with a metabolic specialist, geneticist, or physician specializing in the condition being treated.

Documentation Required

Documentation required for diagnostic confirmation

Documentation is required where noted in the criteria to support molecular testing results or a clinical diagnosis (including family history and urinary orotic acid levels). Acceptable documentation may include chart notes, laboratory tests, and claims records, and/or other information.

Chart notes
Laboratory tests (e.g., urinary orotic acid levels)
Claims records
Molecular genetic testing results showing biallelic pathogenic UMPS variants

Billing Rule

Sample product not acceptable

Receipt of sample product does not satisfy any criteria requirements for coverage.

Background & Evidence

Xuriden is a pyrimidine analog used for uridine replacement and is indicated for the treatment of hereditary orotic aciduria in adults and pediatric patients. Hereditary orotic aciduria (orotic aciduria type 1) is an extremely rare autosomal recessive genetic disorder caused by variants in the UMPS gene resulting in deficiency of uridine 5'-monophosphate synthase and impaired pyrimidine biosynthesis. Clinical features may include megaloblastic anemia, developmental delays, failure to thrive, immunodeficiency, and urinary orotic acid elevation; orotic acid crystals can cause urinary obstruction. Diagnosis is made by detailed patient and family history, clinical evaluation, urine examination, and is most often confirmed by molecular genetic testing (available at specialized laboratories).

Evidence references: Prescribing information: Xuriden oral granules [prescribing information], Wellstat Therapeutics; August 2023.

Documentation required: Documentation is required where noted in the criteria and may include, but is not limited to, chart notes, laboratory tests, claims records, and/or other information to support molecular testing results or clinical diagnosis (including family history and urinary orotic acid levels).

First-degree family relative: defined as parent or sibling; presence of a first-degree family relative with hereditary orotic aciduria is a specified criterion in the clinical diagnostic pathway.

Revision History

2024-12-15selected_revision

Material change: Updated disease name to 'Hereditary Orotic Aciduria (Orotic Aciduria Type 1)' and added requirement for a first‑degree family relative (i.e., parent or sibling) with hereditary orotic aciduria. (is_material)

2025-11-01annual_revisionLatest

Annual revision: Policy title updated from 'Uridine triacetate' to 'Metabolic Disorders - Xuriden' and added documentation guidance stating documentation is required where noted and may include chart notes, laboratory tests, claims records, and/or other information.

Policy Summary

PayerCigna

PolicyMetabolic Disorders - Xuriden

Policy CodePolicy IP0307

Change TypeSelected and Annual Revisions

Effective Date

Next Review Date

SourceLink

On This Page

OpenPayer

Metabolic Disorders - Xuriden

Coverage Summary

Medical Necessity Criteria

Provider Actions

Coding

Background & Evidence

Clinical Thresholds

Revision History