ArticleEuropean journal of neurology2025
Preliminary Observations on the Efficacy of Efgartigimod Therapy in Autoimmune Nodopathy.
Article in European journal of neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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Who cites it
3 citing papers in PubMed.
- Case report: Unilateral optic atrophy and intracranial hypertension in anti-NF155 nodopathy: a case managed with sequential efgartigimod and rituximab.Frontiers in immunology · 2026Article
- Advances in the treatment of autoimmune nodopathy: based on treatment strategies of CIDP.Frontiers in immunology · 2026Review
- Preliminary Observations on the Efficacy of Efgartigimod Therapy in Autoimmune Nodopathy.European journal of neurology · 2025Article
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Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAutoimmune nodopathy exhibits suboptimal responses to conventional immunotherapies. This study investigates the efficacy and safety of efgartigimod, a neonatal Fc receptor blocker, in this condition.
methodsA prospective single-center study enrolled four antibody-confirmed autoimmune nodopathy patients receiving weekly efgartigimod (10 mg/kg) over 4 weeks. Disease progression was assessed using validated neurological scales (INCAT, ISS, I-RODS, and MRC) at baseline (Week 0), weekly during treatment (Weeks 1-4), and 4-week post-treatment follow-up (Week 8).
resultsFour patients (3 females, aged 17-72) responded to efgartigimod within 2 weeks, showing varied improvement based on antibody subtype. Patient 1 (anti-NF186 IgG3+) achieved full remission by Week 2 (INCAT 3 → 0). Patient 2 (anti-NF155 IgG4+) improved progressively (MRC 112 → 119; I-RODS 36 → 40). Patient 3 (anti-NF155 IgG1/IgG4+) quickly stabilized gait in the first week and gradually recovered (INCAT 5 → 2). Patient 4 (anti-CNTN1 IgG1/IgG2/IgG3/IgG4+) reduced tremors rapidly and improved sensorimotor function (ISS 8 → 6; I-RODS 12 → 14) despite a treatment interruption due to a fracture. Antigen-specific efficacy varied: NF186 neuropathy resolved completely, while IgG4-dominant paranodal cases (NF155/CNTN1) partially recovered, prompting sequential B-cell-targeted strategies. No severe adverse events occurred.
conclusionsEfgartigimod provided rapid functional recovery in autoimmune nodopathy. Differential responses by IgG subclass and antigenic targets highlight the necessity for biomarker-guided strategies.
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