Pharmacy Prior Authorization Criteria

Copper Chelating Agents - PA, NF

Indications for Prior Authorization

Cuprimine (penicillamine)

• For diagnosis of Wilson's Disease
  Indicated in the treatment of Wilson's disease.

• For diagnosis of Cystinuria
  Indicated in the treatment of cystinuria.

• For diagnosis of Rheumatoid Arthritis
  Indicated in the treatment of severe, active rheumatoid arthritis in patients who have failed to respond to an adequate trial of conventional therapy.

Syprine (trientine)

• For diagnosis of Wilson's Disease
  Indicated in the treatment of patients with Wilson's disease who are intolerant of penicillamine.

Cuvrior (trientine tetrahydrochloride)

• For diagnosis of Wilson's Disease
  Indicated for the treatment of adult patients with stable Wilson's disease who are de-coppered and tolerant to penicillamine.

Criteria

Brand Cuprimine, generic penicillamine

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Wilson's Disease

• Diagnosis of Wilson's disease (i.e., hepatolenticular degeneration)
AND
• Documentation of one of the following: [5]
• Presence of Kayser-Fleisher rings
• Serum ceruloplasmin (CPN) less than 20 mg/dL
• 24-hour urinary copper excretion greater than 100 mcg
• Liver biopsy with copper dry weight greater than 250 mcg/g
• ATP7B mutation via genetic testing
AND
• Trial and failure, or intolerance to Depen (penicillamine) tablets
AND
• Prescribed by or in consultation with one of the following:
• Gastroenterologist
• Hepatologist

Brand Cuprimine, generic penicillamine

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Cystinuria

• Diagnosis of cystinuria
AND
• Trial and failure, contraindication, or intolerance to both of the following:
• Urinary alkalinization therapy [4]
• Thiola (tiopronin) [A]
AND
• Trial and failure, or intolerance to Depen (penicillamine) tablets
AND
• Prescribed by or in consultation with one of the following:
• Nephrologist
• Urologist

Brand Cuprimine, generic penicillamine

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Rheumatoid Arthritis

• Diagnosis of severe, active rheumatoid arthritis
AND
• Patient's condition is unresponsive to conventional therapy [e.g., traditional DMARDs (e.g., methotrexate, sulfasalazine), TNF inhibitor (e.g., Humira (adalimumab), Enbrel (etanercept)), Non-TNF biologic (e.g., Rinvoq (upadacitinb), Xeljanz (tofacitinib)]
AND
• Trial and failure, or intolerance to Depen (penicillamine) tablets
AND
• Prescribed by or in consultation with a rheumatologist

Brand Cuprimine, generic penicillamine

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)
For diagnosis of Wilson's disease, Cystinuria, Rheumatoid Arthritis

• Patient demonstrates positive clinical response to therapy

Brand Cuprimine, generic penicillamine

Non Formulary

Length of Approval: 12 Month(s)
For diagnosis of Wilson's Disease

• Diagnosis of Wilson's disease (i.e., hepatolenticular degeneration)
AND
• Submission of medical records (e.g., chart notes) documenting one of the following: [5]
• Presence of Kayser-Fleisher rings
• Serum ceruloplasmin (CPN) less than 20 mg/dL
• 24-hour urinary copper excretion greater than 100 mcg
• Liver biopsy with copper dry weight greater than 250 mcg/g
• ATP7B mutation via genetic testing
AND
• Paid claims or submission of medical records (e.g., chart notes) confirming trial and failure, or intolerance to Depen (penicillamine) tablets
AND
• Prescribed by or in consultation with one of the following:
• Gastroenterologist
• Hepatologist

Brand Cuprimine, generic penicillamine

Non Formulary

Length of Approval: 12 Month(s)
For diagnosis of Cystinuria

• Diagnosis of cystinuria
AND
• Paid claims or submission of medical records (e.g., chart notes) confirming trial and failure, contraindication, or intolerance to both of the following:
• Urinary alkalinization therapy [4]
• Thiola (tiopronin) [A]
AND
• Paid claims or submission of medical records (e.g., chart notes) confirming trial and failure, or intolerance to Depen (penicillamine) tablets
AND
• Prescribed by or in consultation with one of the following:
• Nephrologist
• Urologist

Brand Cuprimine, generic penicillamine

Non Formulary

Length of Approval: 12 Month(s)
For diagnosis of Rheumatoid Arthritis

• Diagnosis of severe, active rheumatoid arthritis
AND
• Patient's condition is unresponsive to conventional therapy [e.g., traditional DMARDs (e.g., methotrexate, sulfasalazine), TNF inhibitor (e.g., Humira (adalimumab), Enbrel (etanercept)), Non-TNF biologic (e.g., Rinvoq (upadacitinb), Xeljanz (tofacitinib)]
AND
• Paid claims or submission of medical records (e.g., chart notes) confirming trial and failure, or intolerance to Depen (penicillamine) tablets
AND
• Prescribed by or in consultation with a rheumatologist

Brand Syprine, generic trientine, Cuvrior

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Wilson's disease

• Diagnosis of Wilson's disease (i.e., hepatolenticular degeneration)
AND
• Documentation of one of the following: [5]
• Presence of Kayser-Fleisher rings
• Serum ceruloplasmin (CPN) less than 20 mg/dL
• 24-hour urinary copper excretion greater than 100 mcg
• Liver biopsy with copper dry weight greater than 250 mcg/g
• ATP7B mutation via genetic testing
AND
• Trial and failure, contraindication, or intolerance to Depen (penicillamine) tablets
AND
• For Brand Syprine and Cuvrior, trial and failure, or intolerance to generic trientine
AND
• Prescribed by or in consultation with one of the following:
• Gastroenterologist
• Hepatologist

Brand Syprine, generic trientine, Cuvrior

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)
For diagnosis of Wilson's disease

• Patient demonstrates positive clinical response to therapy
AND
• For Brand Syprine and Cuvrior, trial and failure, or intolerance to generic trientine

Cuvrior, Brand Syprine

Non Formulary

Length of Approval: 12 Month(s)
For diagnosis of Wilson's disease

• Diagnosis of Wilson's disease (i.e., hepatolenticular degeneration)
AND
• Submission of medical records (e.g., chart notes) documenting one of the following: [5]
• Presence of Kayser-Fleisher rings
• Serum ceruloplasmin (CPN) less than 20 mg/dL
• 24-hour urinary copper excretion greater than 100 mcg
• Liver biopsy with copper dry weight greater than 250 mcg/g
• ATP7B mutation via genetic testing
AND
• Paid claims or submission of medical records (e.g., chart notes) confirming trial and failure, contraindication, or intolerance to Depen (penicillamine) tablets
AND
• Paid claims or submission of medical records (e.g., chart notes) confirming trial and failure, or intolerance to generic trientine
AND
• Prescribed by or in consultation with one of the following:
• Gastroenterologist
• Hepatologist

P & T Revisions

2024-10-31, 2024-04-19, 2023-11-07, 2023-09-29, 2023-09-21, 2023-05-26, 2023-04-06, 2022-03-31, 2022-02-09, 2021-12-03, 2021-09-27, 2021-05-20, 2021-03-30, 2020-09-01, 2020-03-24, 2019-12-05, 2019-10-03

References

1. Cuprimine prescribing information. Bausch Health US, LLC. Bridgewater, NJ. October 2020.
2. Syprine prescribing information. Bausch Health US, LLC. Bridgewater, NJ. September 2020.
3. Pearle MS, Goldfarb DS, Assimos DG, et al. Medical management of kidney stones: AUA guideline. J Urol. 2014 Aug;192(2):316-24.
4. Fattah H, Hambaroush Y, Goldfarb DS. Cystine nephrolithiasis. Transl Androl Urol. 2014 Sep 1;3(3):228-233. doi: 10.3978/j.issn.2223-4683.2014.07.04.
5. European Association for Study of Liver. EASL Clinical Practice Guidelines: Wilson's disease. J Hepatol. 2012;56(3):671-685.
6. Cuvrior Prescribing Information. Orphalan SA. Chicago, IL. May 2022.

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End Notes

1. Cystine-binding thiol drugs should be offered to patients with cysteine stones who are unresponsive to dietary modification and urinary alkalinization [3]. Tiopronin should be considered first as it is possibly more effective and associated with fewer adverse events than d-penicillamine.

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Revision History

• 2024-10-31: Added Syprine to non formulary criteria for Wilson's disease.
• 2024-04-19: 2024 Annual Review. No criteria changes. Background updates.
• 2023-11-07: Add generic trientine 500mg capsule as target to guideline.
• 2023-09-29: Program update to standard reauthorization language. No changes to clinical intent.
• 2023-09-21: Added in t/f of generic trientine for Syprine and Cuvrior
• 2023-05-26: Add criteria for Cuvrior. Updated guideline name, background, and references.
• 2023-04-06: Annual Review
• 2022-03-31: Annual Review: no change
• 2022-02-09: Addition of EHB formulary to guideline, no changes to criteria
• 2021-12-03: Updated guideline name, added NF section to Cuprimine (penicillamine) and generic penicillamine
• 2021-09-27: Addition of EHB formulary to guideline, no changes to criteria
• 2021-05-20: Addition of EHB formulary to guideline, no changes to criteria
• 2021-03-30: Annual Review: added tablets when referring to Depen, added examples of conventional therapy for RA, references updated.
• 2020-09-01: Program Update
• 2020-03-24: Guideline Update/Annual Review
• 2019-12-05: Added Clovique (trientine) to existing guideline with criteria to mirror other trientine product
• 2019-10-03: Added generic for Cuprimine (penicillamine)

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