The effect of FcRn inhibition on overlapping autoimmune comorbidity in AChR-seropositive generalized myasthenia gravis.
4 affiliations
- 1Department of Biomedicine, Neuroscience and Advanced Diagnostics (BiND), University of Palermo, Palermo, Italy.
- 2Neurology Unit, Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana, Neuroscience Section, University Hospital "San Giovanni di Dio e Ruggi d'Aragona, Salerno, Italy.
- 3IRCCS SYNLAB- SDN, Napoli, Italy.
- 4Neurology/Stroke Unit, Hospital A. Perrino, Brindisi, Italy.
Abstract
Introduction
Generalized Myasthenia gravis (gMG) is a rare autoimmune disorder of the neuromuscular junction caused by IgG autoantibodies against antigens in the postsynaptic cleft. This study aimed to assess the real-world effectiveness and safety of Efgartigimod (EFG) in AChR-positive gMG with overlapping autoimmune comorbidity.Methods
EFG was administered as 4 consecutive weekly infusions or injections. Myasthenia Gravis Activity of Daily Living (MG-ADL) and Myasthenia Gravis quantitative (QMG) scales were administered at baseline and after 4 weeks. Clinical data on autoimmune comorbidities and prednisone use were collected.Results
35 patients (20 women, mean age 53.6 years) received EFG, with a significant and clinical improvement in both MG-ADL and QMG scores. MG-ADL responder rate (MG-ADL reduction≥2) was 79%, while 68% on QMG (QMG reduction≥3); Minimal symptoms expression was reached in 4 patients, with a mean reduction in daily prednisone dose of 8 mg. Overlapping autoimmune comorbidity was reported in 12 gMG patients (34%). gMG patients with Stiff-Person syndrome, type 1 Diabetes and Lambert-Eaton myasthenic syndrome benefited more from EFG with a higher reduction on MG-ADL.Conclusion
Overlapping autoimmune comorbidity is not rare. EFG demonstrated a meaningful clinical benefit in patients with AChR-positive gMG with overlapping IgG-mediated disorders.
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BibTeX
@article{stefano2026effect,
title = {{The effect of FcRn inhibition on overlapping autoimmune comorbidity in AChR-seropositive generalized myasthenia gravis.}},
author = {Vincenzo Di Stefano and Nicasio Rini and Sofia Campo and Flora D'Amico and Aurora Meli and Simona Maccora and Filippo Brighina and Liliana Bevilacqua and Paolo Barone and Alba Rosa Pati and Mariangela Ferrara and Augusto Maria Rini and Claudia Vinciguerra},
journal = {Frontiers in neurology},
year = {2026},
month = oct,
doi = {10.3389/fneur.2026.1919993},
url = {https://europepmc.org/article/MED/42903245},
note = {CC BY},
}
RIS
TY - JOUR
TI - The effect of FcRn inhibition on overlapping autoimmune comorbidity in AChR-seropositive generalized myasthenia gravis.
AU - Vincenzo Di Stefano
AU - Nicasio Rini
AU - Sofia Campo
AU - Flora D'Amico
AU - Aurora Meli
AU - Simona Maccora
AU - Filippo Brighina
AU - Liliana Bevilacqua
AU - Paolo Barone
AU - Alba Rosa Pati
AU - Mariangela Ferrara
AU - Augusto Maria Rini
AU - Claudia Vinciguerra
PY - 2026
DA - 2026/10/06
JO - Frontiers in neurology
DO - 10.3389/fneur.2026.1919993
UR - https://europepmc.org/article/MED/42903245
AB - Introduction Generalized Myasthenia gravis (gMG) is a rare autoimmune disorder of the neuromuscular junction caused by IgG autoantibodies against antigens in the postsynaptic cleft. This study aimed to assess the real-world effectiveness and safety of Efgartigimod (EFG) in AChR-positive gMG with overlapping autoimmune comorbidity.Methods EFG was administered as 4 consecutive weekly infusions or injections. Myasthenia Gravis Activity of Daily Living (MG-ADL) and Myasthenia Gravis quantitative (QMG) scales were administered at baseline and after 4 weeks. Clinical data on autoimmune comorbidities and prednisone use were collected.Results 35 patients (20 women, mean age 53.6 years) received EFG, with a significant and clinical improvement in both MG-ADL and QMG scores. MG-ADL responder rate (MG-ADL reduction≥2) was 79%, while 68% on QMG (QMG reduction≥3); Minimal symptoms expression was reached in 4 patients, with a mean reduction in daily prednisone dose of 8 mg. Overlapping autoimmune comorbidity was reported in 12 gMG patients (34%). gMG patients with Stiff-Person syndrome, type 1 Diabetes and Lambert-Eaton myasthenic syndrome benefited more from EFG with a higher reduction on MG-ADL.Conclusion Overlapping autoimmune comorbidity is not rare. EFG demonstrated a meaningful clinical benefit in patients with AChR-positive gMG with overlapping IgG-mediated disorders.
SN - 1664-2295
ER -