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.

Rossini, E., Di Stefano, V., Iorio, R., Habetswallner, F., Maestri, M., Vinciguerra, C., Pennisi, E. M., Di Martino, G., Rini, N., Falso, S., Marini, S., Ricciardi, D., Guida, M., Morino, S., Garibaldi, M., Leonardi, L., Marando, D., Tufano, L., Antonini, G., Fionda, L., Ravulizumab for generalized Myasthenia Gravis: a multicenter real-life experience, <<JOURNAL OF NEUROLOGY>>, 2025; 272 (6): N/A-N/A. [doi:10.1007/s00415-025-13127-8] [https://hdl.handle.net/10807/316318]

Ravulizumab for generalized Myasthenia Gravis: a multicenter real-life experience

Iorio, Raffaele;Falso, Silvia;Marini, Sofia;
2025

Abstract

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.
2025
Inglese
Rossini, E., Di Stefano, V., Iorio, R., Habetswallner, F., Maestri, M., Vinciguerra, C., Pennisi, E. M., Di Martino, G., Rini, N., Falso, S., Marini, S., Ricciardi, D., Guida, M., Morino, S., Garibaldi, M., Leonardi, L., Marando, D., Tufano, L., Antonini, G., Fionda, L., Ravulizumab for generalized Myasthenia Gravis: a multicenter real-life experience, <<JOURNAL OF NEUROLOGY>>, 2025; 272 (6): N/A-N/A. [doi:10.1007/s00415-025-13127-8] [https://hdl.handle.net/10807/316318]
File in questo prodotto:
File Dimensione Formato  
s00415-025-13127-8.pdf

accesso aperto

Licenza: Creative commons
Dimensione 1.01 MB
Formato Adobe PDF
1.01 MB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10807/316318
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 0
  • ???jsp.display-item.citation.isi??? 0
social impact