CurrentCignaPolicy N/A

Chelating Agents - Trientine Products Prior Authorization Policy

This policy governs prior authorization requirements and medical necessity criteria for trientine products (Cuvrior and Syprine) for members covered under Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyChelating Agents - Trientine Products Prior Authorization Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionObtain prior authorization and document confirmed Wilson's disease plus justification for trientine over penicillamine.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

2trientine products covered

1approval duration (years)

3specialist types

3non-covered indications

Coverage Criteria for Trientine Products

FDA-Approved indication (Wilson's disease)

Approve for 1 year if ALL of the following are met:

Root: Approve for 1 year if ALL of the following (A, B, and C) are met

A: Diagnosis confirmed: Diagnosis of Wilson's disease is confirmed by ONE of: (i) genetic testing showing biallelic pathogenic ATP7B mutations; OR (ii) confirmation of at least TWO of: (a) presence of Kayser-Fischer rings; (b) serum ceruloplasmin < 20 mg/dL; (c) liver biopsy findings consistent with Wilson's disease; (d) 24-hour urinary copper > 40 mcg/24 hours.

B: Justification for trientine instead of penicillamine: Patient meets ONE of: (i) tried one penicillamine product and is intolerant to penicillamine therapy; OR (ii) has clinical features indicating potential for intolerance to penicillamine therapy (examples: history of renal disease, congestive splenomegaly causing severe thrombocytopenia, autoimmune tendency); OR (iii) has a contraindication to penicillamine therapy; OR (iv) has neurologic manifestations of Wilson's disease; OR (v) is pregnant; OR (vi) has already started therapy with trientine (Cuvrior or Syprine, generic).

Policy Summary

PayerCigna

PolicyChelating Agents - Trientine Products Prior Authorization Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionObtain prior authorization and document confirmed Wilson's disease plus justification for trientine over penicillamine.

SourceLink

On This Page

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Provider Requirements and Prior Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required for trientine products (Cuvrior™, Syprine® and generics). All approvals are provided for the duration noted in the policy (approve for 1 year when criteria are met). Because of the specialized evaluation and monitoring required, therapy must be prescribed by or in consultation with a gastroenterologist, hepatologist, or liver transplant physician.

Affected products: Cuvrior™ (trientine tetrahydrochloride tablets), Syprine® (trientine hydrochloride capsules) and generics.
Approval duration: 1 year when medical necessity criteria are met.

Step Therapy

Step Therapy / Trial Requirement

Step therapy / trial requirement: Document prior trial and intolerance to penicillamine OR provide an allowed justification. Acceptable justifications include: intolerance to penicillamine after trial, clinical features indicating potential for intolerance (for example history of renal disease, congestive splenomegaly with severe thrombocytopenia, autoimmune tendency), a contraindication to penicillamine, neurologic manifestations of Wilson's disease, pregnancy, or that the patient has already been started on trientine therapy.

If claiming prior penicillamine intolerance, note the penicillamine product tried (examples: Cuprimine, Depen).
Allowed alternatives to a prior penicillamine trial: documented clinical features indicating likely intolerance, documented contraindication, neurologic disease, pregnancy, or current trientine initiation.

Documentation Required

Required Documentation

Required documentation must support a confirmed diagnosis of Wilson's disease and the prescriber specialty/treatment history. Confirm diagnosis with genetic testing (biallelic pathogenic ATP7B mutations) OR at least two clinical features (Kayser-Fleischer rings; serum ceruloplasmin < 20 mg/dL; liver biopsy consistent with Wilson's disease; 24-hour urinary copper > 40 mcg/24 hr). Also document the prescriber is a gastroenterologist, hepatologist, or liver transplant physician, or that the treatment is prescribed in consultation with one. Include documentation of prior therapies, intolerance or contraindications to penicillamine when applicable.

Diagnostic evidence: genetic testing OR ≥2 clinical features as listed above.
Prescriber requirement: prescribing or consulting specialist (gastroenterology, hepatology, liver transplant).
Treatment history: documentation of prior penicillamine trial and intolerance, or stated rationale if a trial was not attempted.

Definitions and Product Details

inv-09: Trientine products

Syprine (trientine hydrochloride)Capsule formulation; indicated for treatment of patients with Wilson's disease who are intolerant of penicillamine; content differs from other trientine products (not mg-for-mg interchangeable).

Cuvrior (trientine tetrahydrochloride)Tablet formulation; indicated for treatment of adults with stable, de-coppered Wilson's disease who are tolerant to penicillamine.

Generic trientine formulationsAvailable as generic capsules (trientine hydrochloride) and tablets; product content differs between products and they are not interchangeable on a mg-per-mg basis.

inv-10: Wilson's disease diagnostic criteria

Genetic confirmationGenetic testing showing biallelic pathogenic ATP7B mutations confirms diagnosis of Wilson's disease (acceptable for symptomatic or asymptomatic individuals).

Clinical criteria — Kayser-Fleischer ringsPresence of Kayser-Fleischer rings is one of the clinical features used; diagnosis can be confirmed with this plus at least one other listed feature (two required if genetic testing not available).

Clinical criteria — low ceruloplasminSerum ceruloplasmin level < 20 mg/dL is an accepted clinical feature contributing to diagnosis (requires a second feature unless genetic confirmation present).

Clinical criteria — liver biopsyLiver biopsy findings consistent with Wilson's disease may be used as one of the clinical features to meet diagnostic criteria (two features required if no genetic confirmation).

Clinical criteria — 24-hour urinary copper24-hour urinary copper > 40 mcg/24 hours is an accepted clinical feature for diagnosis and may be used alongside another feature to confirm Wilson's disease.

OpenPayer

Chelating Agents - Trientine Products Prior Authorization Policy

Coverage Criteria for Trientine Products

Provider Requirements and Prior Authorization

Definitions and Product Details

Background