CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Onpattro (patisiran) coverage criteria — hATTR amyloidosis with polyneuropathy

Clinical coverage criteria for intravenous Onpattro (patisiran) for treatment of hATTR amyloidosis with polyneuropathy for Neighborhood Health Plan of Rhode Island members (Medicaid, Commercial, Medicare-Medicaid Plan).

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyOnpattro (patisiran) coverage criteria — hATTR amyloidosis with polyneuropathy

Policy CodePolicy N/A

Change TypeNo material change

Effective DateApr 10, 2019

Next Review DateN/A

Key ActionObtain prior authorization with documentation of definitive hATTR diagnosis (biopsy plus pathogenic TTR variant) and baseline objective neuropathy measures.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsinitial authorization length

300 unitsmax units / interval

J0222HCPCS

E85.1diagnosis (ICD-10)

>=18min age

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria for Onpattro (patisiran)

Initial Approval Criteria

Covered when ALL of the following are met:

Initial approval requirements: Member is at least 18 years of age; AND Member is receiving supplementation with vitamins at the recommended daily allowance; AND Must not be used in combination with other transthyretin (TTR)-reducing or stabilizing agents (e.g., eplontersen, vutisiran, acoramidis, tafamidis) unless documented inadequate response, intolerance, or contraindication to monotherapy when treating overlapping ATTR-CM and ATTR-PN; AND Must be prescribed by or in consultation with a neurologist or physician specializing in amyloidosis related to hATTR; AND Member has a definitive diagnosis of hATTR amyloidosis documented by tissue biopsy demonstrating amyloid deposition and identification of a pathogenic TTR variant by molecular genetic testing; AND Member has polyneuropathy demonstrated by at least TWO of: subjective symptoms suggestive of neuropathy; abnormal nerve conduction studies consistent with polyneuropathy; abnormal neurologic examination suggestive of neuropathy; AND Peripheral neuropathy is attributed to hATTR with other causes of neuropathy excluded; AND Baseline objective measurement of strength/weakness is documented (e.g., MRC muscle strength, mNIS+7); AND Dosing consistent with guideline: 0.3 mg/kg IV every 3 weeks (or 30 mg IV every 3 weeks for weight >= 100 kg).

Renewal Criteria

Coverage may be renewed when ALL of the following are met:

Renewal requirements: Member continues to meet the universal and indication-specific criteria; AND Absence of unacceptable toxicity (examples include severe infusion-related reactions, ocular symptoms related to hypovitaminosis A); AND Evidence of disease response or stabilization compared to pre-treatment baseline, as shown by stabilization or improvement in symptoms/signs (e.g., ambulation, neurologic symptom burden, activities of daily living) or objective measures (e.g., MRC, mNIS+7, Norfolk QoL-DN).

Coverage will not be provided for members with the following conditions: prior or planned liver transplant; severe renal impairment or end-stage renal disease; New York Heart Association (NYHA) heart failure classification > 2; other known causes of neuropathy (for example, uncontrolled diabetes or sensorimotor/autonomic neuropathy not related to hATTR amyloidosis); primary or leptomeningeal amyloidosis; and cardiomyopathy due to hATTR (hATTR-CM).

Billing and Diagnosis Codes

Covered HCPCS CodesHCPCSCovered

J0222

Injection, patisiran, 0.1 mg; 1 billable unit = 0.1 mg

NDCNDC

71336-1000-xx

Onpattro 10 mg/5 mL single-dose vial

Covered Diagnosis CodesICD-10Covered

E85.1

Neuropathic heredofamilial amyloidosis

inv-07: Max HCPCS units per dosing interval

Maximum HCPCS billing units300 billable units (equivalent to 30 mg) per dosing interval

Dosing intervalEvery 3 weeks

HCPCS unit definition1 billable unit = 0.1 mg (see HCPCS code J0222 for billing)

Dose cap exampleStandard adult dose equivalent: 30 mg IV every 3 weeks (300 units)

Provider Requirements and Actions

Prior Authorization

Prior Authorization and Dosing

Prior authorization is required. Member must be at least 18 years of age. Dosing authorization will follow recommended dosing: for hATTR polyneuropathy, administer 0.3 mg/kg IV every 3 weeks for weight < =100 kg; administer 30 mg IV every 3 weeks for weight ≥100 kg. HCPCS J0222 (patisiran 0.1 mg) applies for billing.

Prior authorization required
Age minimum: 18 years
Dosing: 0.3 mg/kg IV q3 weeks for weight <100 kg; 30 mg IV q3 weeks for weight ≥100 kg
Billing code: HCPCS J0222 (0.1 mg units)

Step Therapy

Combination Therapy / Step Requirement

Do not use in combination with other transthyretin (TTR)–reducing or TTR–stabilizing agents. If treating overlapping ATTR-CM and ATTR-PN with combination therapy (e.g., patisiran plus a TTR stabilizer such as acoramidis or tafamidis), combination will only be considered when there is documented inadequate response, intolerance, or contraindication to monotherapy with the alternative agent (e.g., vutisiran).

Prohibited concomitant agents: eplontersen (Wainua), vutisiran (Amvuttra), acoramidis (Acoramidis/Attruby), tafamidis (Vyndaqel/Vyndamax)
Combination therapy allowed only after documented inadequate response, intolerance, or contraindication to appropriate monotherapy

Documentation Required

Required Diagnostic Documentation

Required diagnostic documentation must be submitted with the prior authorization: definitive diagnosis of hereditary transthyretin (hATTR) amyloidosis documented by tissue biopsy showing amyloid deposition plus molecular genetic testing identifying a pathogenic TTR variant; documentation that peripheral neuropathy is due to hATTR with other causes excluded; and objective baseline measures of neuropathy (e.g., MRC muscle strength, mNIS+7). The prescriber must be a neurologist or a physician specializing in amyloidosis or prescribe in consultation with such a specialist. Vitamin supplementation at recommended daily allowance should be documented.

Tissue biopsy confirming amyloid deposition
Molecular genetic testing identifying pathogenic TTR variant
Documentation of ≥2 findings consistent with polyneuropathy (symptoms, nerve conduction studies, neurological exam)
Objective baseline neuropathy score (e.g., MRC, mNIS+7)
Prescriber: neurologist or specialist in amyloidosis (or documented consultation)
Documentation of vitamin supplementation at recommended daily allowance

Background

Hereditary transthyretin-mediated (hATTR) amyloidosis is a systemic, genetic disorder in which misfolded transthyretin (TTR) forms amyloid deposits that can cause progressive peripheral polyneuropathy. Patisiran (Onpattro) is an RNA interference therapeutic that reduces hepatic production of transthyretin, and has been studied for improvement in neuropathy and related functional and nutritional measures in affected patients.

For treatment consideration, the drug must be prescribed by or in consultation with a neurologist or a physician specializing in amyloidosis, and the member must have a definitive diagnosis confirmed by tissue biopsy demonstrating amyloid deposition plus identification of a pathogenic TTR variant by molecular genetic testing. Clinical evidence of polyneuropathy is required, demonstrated by at least two of: subjective neuropathic symptoms, abnormal nerve conduction studies consistent with polyneuropathy, or an abnormal neurologic examination; other causes of neuropathy must be excluded and baseline objective strength/weakness measurement (e.g., MRC or mNIS+7) should be documented.

Definitions

inv-13: hATTR amyloidosis with polyneuropathy

DefinitionHereditary transthyretin-mediated (hATTR) amyloidosis with polyneuropathy: hATTR confirmed by tissue biopsy demonstrating amyloid deposition plus identification of a pathogenic TTR variant by molecular genetic testing

Clinical manifestation requiredPresence of polyneuropathy attributed to hATTR with other causes of neuropathy excluded

Diagnostic evidence for neuropathyPolyneuropathy must be demonstrated by at least TWO of: subjective symptoms suggestive of neuropathy, abnormal nerve conduction studies consistent with polyneuropathy, or abnormal neurologic examination suggestive of neuropathy

Baseline assessmentDocumentation of baseline objective strength/weakness measurement (e.g., MRC, mNIS+7) is required

Prescriber requirementMust be prescribed by or in consultation with a neurologist or physician specializing in treatment of amyloidosis related to hATTR