TY - JOUR
T1 - Ravulizumab for generalized Myasthenia Gravis: a multicenter real-life experience
AU - Rossini, Elena
AU - Di Stefano, Vincenzo
AU - Iorio, Raffaele
AU - Habetswallner, Francesco
AU - Maestri, Michelangelo
AU - Vinciguerra, Claudia
AU - Pennisi, Elena Maria
AU - Di Martino, Giuseppe
AU - Rini, Nicasio
AU - Falso, Silvia
AU - Marini, Sofia
AU - Ricciardi, Dario
AU - Guida, Melania
AU - Morino, Stefania
AU - Garibaldi, Matteo
AU - Leonardi, Luca
AU - Marando, Demetrio
AU - Tufano, Laura
AU - Antonini, Giovanni
AU - Fionda, Laura
PY - 2025
Y1 - 2025
N2 - Introduction: Ravulizumab, a monoclonal antibody targeting C5, was recently approved for the treatment of anti-AChR positive generalized myasthenia gravis (gMG) patients. The objective of this study is to present the Italian multicenter real-world experience evaluating the safety and efficacy of ravulizumab in gMG within the context of the Expanded Early Access Program (EAP). Methods: We conducted a retrospective study in 7 gMG referral centres in Italy. Demographic and clinical characteristics were recorded at baseline and during follow-up through clinical scale changes including Myasthenia Gravis-Activities of Daily Living (MG-ADL), Quantitative Myasthenia Gravis (QMG) and Myasthenia Gravis Composite (MGC). Frequency of minimal symptom expression (MSE) and changes in concomitant medications were also evaluated. Results: Twenty-four gMG patients (10/24 females) aged between 24 and 82 years (Median 60.5, IQR 52.5–67.5), were included. Fifteen patients had undergone thymectomy, and 14 had a thymoma. Median follow-up duration was 26 weeks (range 10–74, IQR 26–42). MG-ADL and QMG scores showed a significant decrease with respect to baseline (p < 0.001). MSE was achieved by 37.5% patients at the last available follow-up. Tapering of prednisone daily dosage was possible in 76% of patients. Thymoma was significantly associated with QMG score reduction and the frequency of QMG responders at week 2 (p = 0.03). Three patients discontinued treatment. One patient experienced a myasthenic exacerbation and needed rescue therapy. Infectious adverse events were reported in 5/24 patients, and a Stevens-Johnson syndrome in one patient. Conclusions: Real-world data confirm the effectiveness, safety, and prednisone-sparing effect of ravulizumab in patients with gMG, especially in those with thymoma.
AB - Introduction: Ravulizumab, a monoclonal antibody targeting C5, was recently approved for the treatment of anti-AChR positive generalized myasthenia gravis (gMG) patients. The objective of this study is to present the Italian multicenter real-world experience evaluating the safety and efficacy of ravulizumab in gMG within the context of the Expanded Early Access Program (EAP). Methods: We conducted a retrospective study in 7 gMG referral centres in Italy. Demographic and clinical characteristics were recorded at baseline and during follow-up through clinical scale changes including Myasthenia Gravis-Activities of Daily Living (MG-ADL), Quantitative Myasthenia Gravis (QMG) and Myasthenia Gravis Composite (MGC). Frequency of minimal symptom expression (MSE) and changes in concomitant medications were also evaluated. Results: Twenty-four gMG patients (10/24 females) aged between 24 and 82 years (Median 60.5, IQR 52.5–67.5), were included. Fifteen patients had undergone thymectomy, and 14 had a thymoma. Median follow-up duration was 26 weeks (range 10–74, IQR 26–42). MG-ADL and QMG scores showed a significant decrease with respect to baseline (p < 0.001). MSE was achieved by 37.5% patients at the last available follow-up. Tapering of prednisone daily dosage was possible in 76% of patients. Thymoma was significantly associated with QMG score reduction and the frequency of QMG responders at week 2 (p = 0.03). Three patients discontinued treatment. One patient experienced a myasthenic exacerbation and needed rescue therapy. Infectious adverse events were reported in 5/24 patients, and a Stevens-Johnson syndrome in one patient. Conclusions: Real-world data confirm the effectiveness, safety, and prednisone-sparing effect of ravulizumab in patients with gMG, especially in those with thymoma.
KW - Complement inhibitor therapy
KW - Myasthenia Gravis
KW - Ravulizumab
KW - Real life study
KW - Complement inhibitor therapy
KW - Myasthenia Gravis
KW - Ravulizumab
KW - Real life study
UR - https://publicatt.unicatt.it/handle/10807/316318
UR - https://www.scopus.com/inward/citedby.uri?partnerID=HzOxMe3b&scp=105005220917&origin=inward
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=105005220917&origin=inward
U2 - 10.1007/s00415-025-13127-8
DO - 10.1007/s00415-025-13127-8
M3 - Article
SN - 0340-5354
VL - 272
SP - N/A-N/A
JO - Journal of Neurology
JF - Journal of Neurology
IS - 6
ER -