CurrentViva HealthPolicy IC-0401

Tegsedi (inotersen) coverage for hATTR polyneuropathy

Defines prior authorization, coverage criteria, dosing, and renewal requirements for Tegsedi (inotersen) for treatment of hereditary transthyretin-mediated (hATTR) amyloid polyneuropathy for Viva Health members.

Policy Summary

PayerViva Health

PolicyTegsedi (inotersen) coverage for hATTR polyneuropathy

Policy CodePolicy IC-0401

Change TypeNo material changes

Effective Date

Next Review Date

Key ActionObtain prior authorization for an initial 6-month approval and ensure documentation of definitive hATTR diagnosis, baseline labs (platelets ≥100 x10^9/L, UPCR <1000 mg/g), and absence of concurrent TTR-reducing agents.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

284 mgprescribed dose

6 moinitial prior authorization length

≥18minimum age

≥100platelet requirement

<1000UPCR requirement

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Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met

Age and general requirements: Patient is at least 18 years of age

Universal baseline and monitoring: Patient has a platelet count ≥100 x10^9/L; baseline urine protein-to-creatinine ratio (UPCR) <1000 mg/g with UPCR monitored every 2 weeks during treatment; patient is receiving vitamin A supplementation; drug is not used in combination with other transthyretin (TTR)-reducing or -stabilizing agents (e.g., patisiran, tafamidis, vutrisiran, eplontersen)

Diagnosis and neuropathy documentation: Definitive diagnosis of hATTR amyloidosis documented by imaging or histopathology and a heterozygous pathogenic or likely pathogenic TTR variant on molecular genetic testing; polyneuropathy demonstrated by at least TWO of: subjective patient symptoms suggestive of neuropathy; abnormal nerve conduction studies consistent with polyneuropathy; abnormal neurologic examination suggestive of neuropathy; peripheral neuropathy attributed to hATTR with other causes excluded; baseline strength documented with an objective tool (e.g., MRC); patient has not received an orthotopic liver transplant (OLT)

Initial therapy — initial coverage criteria

Covered when ALL of the following are met

Age: Patient is at least 18 years of age

Baseline labs and supplements: Platelet count ≥100 x10^9/L; baseline UPCR <1000 mg/g with monitoring every 2 weeks during treatment; patient receiving vitamin A supplementation; not used concurrently with other TTR-reducing or -stabilizing agents

Diagnosis and neuropathy: Definitive hATTR diagnosis with heterozygous pathogenic/likely pathogenic TTR variant and supportive amyloidosis evidence; neuropathy documented by at least two findings (subjective symptoms, abnormal nerve conduction studies, or abnormal neurologic exam); other causes excluded; baseline objective strength documented; no prior orthotopic liver transplant (OLT)

Continuation/Renewal — Renewal may be approved when ALL of the following are met

Renewal may be approved when ALL of the following are met

Continued eligibility: Patient continues to meet the universal and indication-specific initial criteria

Safety: Absence of unacceptable toxicity from the drug (examples include: stroke, cervicocephalic arterial dissection, ocular symptoms related to vitamin A deficiency such as night blindness, severe thrombocytopenia, glomerulonephritis/renal toxicity, liver injury, serious inflammatory or immune adverse reactions, hypersensitivity/antibody formation)

Response: Disease response compared to pre-treatment baseline as evidenced by stabilization or improvement in one or more of: signs and symptoms of neuropathy, MRC muscle strength

Coverage excludes use of Tegsedi in combination with other transthyretin (TTR)–reducing or stabilizing agents. Examples include patisiran, tafamidis, vutrisiran, and eplontersen; concurrent use with these agents is specifically prohibited and will result in non‑approval. Additionally, patients who have received an orthotopic liver transplant (OLT) are not eligible for coverage.

Patients do not meet initial coverage criteria if baseline laboratory values fall outside required thresholds. Specifically, a baseline platelet count < 100 x10^9/L or a baseline urine protein-to-creatinine ratio (UPCR) ≥ 1000 mg/g will render the request not medically necessary. For covered patients, UPCR must be < 1000 mg/g at baseline and then monitored every 2 weeks during treatment; baseline vitamin A supplementation is also required.

Coding and Clinical Thresholds

HCPCSHCPCS

J3490	Unclassified drugs
C9399	Unclassified drugs or biologicals

NDCNDC

72126-0007-xx

Tegsedi 284 mg/1.5 mL single-dose prefilled syringe NDC

Covered ICD-10 Diagnosis CodesICD-10Covered

E85.1

Neuropathic heredofamilial amyloidosis

inv-08: Platelet count — baseline requirement

Baseline platelet requirementPatient must have a platelet count ≥ 100 x 10^9/L prior to initiation

Action if below thresholdPatients with baseline platelet count < 100 x 10^9/L do not meet initial criteria and are not eligible for initiation

Monitoring implicationPlatelet count is a required baseline laboratory prior to starting therapy

inv-09: UPCR baseline and monitoring — baseline threshold and monitoring frequency

Baseline UPCR thresholdBaseline urine protein-to-creatinine ratio (UPCR) must be < 1000 mg/g

Monitoring frequencyUPCR must be monitored every 2 weeks during treatment

Action if above thresholdPatients with baseline UPCR ≥ 1000 mg/g do not meet initial criteria

inv-10: Dose — prescribed dose

Prescribed dose284 mg administered subcutaneously once weekly

Unit/intervalOne dose of 284 mg every 7 days

Quantity limit referenceMaximum units per dose/time defined as 284 mg every 7 days

Provider Requirements and Operational Notes

Prior Authorization

Prior Authorization Required

Prior authorization is required for initiation and continuation of therapy. Initial approvals are granted for 6 months. Renewals may be authorized annually thereafter. Providers should submit a renewal request with supporting documentation of ongoing benefit.

Initial approval length: 6 months
Renewal: may be renewed annually

Denial Risk

Concurrent Therapy Exclusion

Do not initiate or continue this therapy in combination with other transthyretin (TTR) reducing or stabilizing agents. Concurrent use with agents such as patisiran, tafamidis, vutrisiran, eplontersen, or other TTR-targeting therapies is prohibited and may result in denial of coverage.

Concurrent therapy exclusion: No combination use with other TTR-reducing or stabilizing agents (e.g., patisiran, tafamidis, vutrisiran, eplontersen)

Documentation Required

Required Diagnostic Documentation

Documentation must confirm a definitive diagnosis of hereditary transthyretin-mediated (hATTR) amyloidosis. Provide molecular genetic testing results demonstrating a heterozygous pathogenic or likely pathogenic TTR variant and supportive clinical or diagnostic evidence (imaging or histopathology) consistent with amyloidosis. For polyneuropathy indication, include objective findings consistent with neuropathy and documentation that other causes have been excluded.

Definitive diagnosis: proband with suggestive findings (imaging or histopathology) plus heterozygous pathogenic/likely pathogenic TTR variant on molecular genetic testing
Polyneuropathy documentation: at least two of - suggestive subjective symptoms, abnormal nerve conduction studies, or abnormal neurological exam
Exclude alternative causes of neuropathy and document baseline strength using an objective tool (e.g., MRC muscle strength)
Confirm patient has not received orthotopic liver transplant (OLT)

Documentation Required

Required Baseline Labs and Monitoring

Obtain baseline laboratory and urine studies prior to initiation and monitor per schedule. Ensure platelet count and urine protein to creatinine ratio (UPCR) meet the criteria and are followed during treatment.

Baseline platelet count: >= 100 x 10^9/L
Baseline UPCR: < 1000 mg/g
UPCR monitoring: every 2 weeks during treatment
Vitamin A supplementation: patient must be receiving recommended daily allowance

Initial Therapy — Detailed Criteria

Initial therapy — initial coverage criteria

Covered when ALL of the following are met

Age and general requirements: Patient is at least 18 years of age

Universal baseline and monitoring: Patient has a platelet count ≥100 x10^9/L; baseline UPCR <1000 mg/g with UPCR monitored every 2 weeks during treatment; patient is receiving vitamin A supplementation; must not be used in combination with other TTR-reducing or -stabilizing agents (e.g., patisiran, tafamidis, vutrisiran, eplontersen)

Diagnosis and neuropathy documentation: Definitive diagnosis of hATTR amyloidosis with imaging or histopathology and heterozygous pathogenic/likely pathogenic TTR variant; polyneuropathy demonstrated by at least two of: subjective symptoms, abnormal nerve conduction studies, or abnormal neurologic exam; peripheral neuropathy attributed to hATTR with other causes excluded; baseline strength documented with an objective tool (e.g., MRC); patient has not been the recipient of an orthotopic liver transplant (OLT)

Background

Hereditary transthyretin‑mediated (hATTR) amyloidosis is a genetic disorder causing progressive polyneuropathy due to pathogenic variants in the TTR gene. Tegsedi (inotersen) is indicated for treatment of the polyneuropathy of hATTR and requires specific diagnostic confirmation and laboratory monitoring. Covered patients must have a definitive diagnosis of hATTR—documented by imaging or histopathology consistent with amyloidosis plus a heterozygous pathogenic or likely pathogenic TTR variant—and demonstrate polyneuropathy by at least two findings (for example, suggestive symptoms, abnormal nerve conduction studies, or abnormal neurologic exam). Baseline objective strength measurement must be documented, other causes of neuropathy excluded, and baseline laboratory thresholds met, including platelets ≥ 100 x10^9/L and UPCR < 1000 mg/g with UPCR monitoring every 2 weeks. Tegsedi must not be used in combination with other TTR‑reducing or stabilizing agents.

OpenPayer

Tegsedi (inotersen) coverage for hATTR polyneuropathy

Coverage Criteria for Tegsedi (inotersen)

Coding and Clinical Thresholds

Provider Requirements and Operational Notes

Initial Therapy — Detailed Criteria

Continuation / Renewal Criteria

Quantity and Dispensing Limits

Site-of-Care Guidance

Definitions

Background