journal article
Open Access
Jul 01, 2025
Preliminary Observations on the Efficacy of Efgartigimod Therapy in Autoimmune Nodopathy
Abstract
ABSTRACTBackgroundAutoimmune 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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Details
- Published
- Jul 01, 2025
- Vol/Issue
- 32(7)
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Cite This Article
Hongfei Tai, Songtao Niu, Hua Pan, et al. (2025). Preliminary Observations on the Efficacy of Efgartigimod Therapy in Autoimmune Nodopathy. European Journal of Neurology, 32(7). https://doi.org/10.1111/ene.70269
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