Eplontersen for Hereditary Transthyretin Amyloidosis With Polyneuropathy: An Exploratory Analysis of Treatment Effect in Male and Female Patients

Muscle Nerve. 2026 Jun;73(6):1118-1127. doi: 10.1002/mus.70230. Epub 2026 Apr 6.

Abstract

Introduction/aims: Eplontersen is approved in multiple regions for adults with hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN). This exploratory analysis was conducted to evaluate the treatment effect of eplontersen by sex in patients with ATTRv-PN from the NEURO-TTRansform trial (NCT04136184).

Methods: Participants in NEURO-TTRansform who received ≥ 1 dose of eplontersen were included in this analysis; participants in the inotersen reference group who switched to eplontersen were excluded. Eplontersen was evaluated in the overall patient population and in a subgroup with cardiomyopathy. Change from baseline in the NEURO-TTRansform primary endpoints of serum transthyretin (TTR) levels to Week 65, modified Neuropathy Impairment Score +7 (mNIS+7) composite score to Week 66, and Norfolk Quality of Life-Diabetic Neuropathy (Norfolk QoL-DN) total score to Week 66 were evaluated. A historical placebo group from the NEURO-TTR trial (NCT01737398) was included as a control.

Results: The eplontersen group comprised 100 (69.4%) male and 44 (30.6%) female patients. The placebo group comprised 41 (68.3%) male and 19 (31.7%) female patients. For both sexes, treatment with eplontersen decreased serum TTR levels by > 80% (vs. reductions of 3%-13% with placebo), maintained mNIS+7 scores, and improved Norfolk QoL-DN scores (vs. deterioration with placebo). The treatment effect of eplontersen was similar in male and female patients with cardiomyopathy.

Discussion: Eplontersen halted neuropathy impairment and improved quality of life versus placebo to a similar degree in both male and female patients with ATTRv-PN, including those with cardiomyopathy. These findings support the use of eplontersen as an effective treatment for ATTRv-PN regardless of sex.

Keywords: ATTRv‐PN; eplontersen; female; male; quality of life.

Publication types

  • Randomized Controlled Trial

MeSH terms

  • Adult
  • Aged
  • Amyloid Neuropathies, Familial* / complications
  • Amyloid Neuropathies, Familial* / drug therapy
  • Female
  • Humans
  • Male
  • Middle Aged
  • Oligonucleotides* / therapeutic use
  • Polyneuropathies* / drug therapy
  • Prealbumin / metabolism
  • Quality of Life
  • Sex Characteristics
  • Sex Factors
  • Treatment Outcome

Substances

  • Prealbumin
  • Oligonucleotides
  • eplontersen

Supplementary concepts

  • Amyloidosis, Hereditary, Transthyretin-Related

Associated data

  • ClinicalTrials.gov/NCT01737398
  • ClinicalTrials.gov/NCT04136184