Frontiers in neurology · 2026
The effect of FcRn inhibition on overlapping autoimmune comorbidity in AChR-seropositive generalized myasthenia gravis.
СтатьяНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Di Stefano Vincenzo, Rini Nicasio, Campo Sofia, D'Amico Flora, Meli Aurora, Maccora Simona, Brighina Filippo, Bevilacqua Liliana, Barone Paolo, Pati Alba Rosa, Ferrara Mariangela, Rini Augusto Maria, Vinciguerra Claudia
Аннотация
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.
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.