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Treatment of refractory myasthenia gravis by double‐filtration plasmapheresis and rituximab: A case series of nine patients and literature review
Author(s) -
Bennani Hamza N.,
Lagrange Emmeline,
Noble Johan,
Malvezzi Paolo,
Motte Lionel,
Chevallier Eloi,
Rostaing Lionel,
Jouve Thomas
Publication year - 2021
Publication title -
journal of clinical apheresis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.697
H-Index - 46
eISSN - 1098-1101
pISSN - 0733-2459
DOI - 10.1002/jca.21868
Subject(s) - medicine , plasmapheresis , myasthenia gravis , rituximab , refractory (planetary science) , series (stratigraphy) , surgery , pediatrics , antibody , immunology , physics , astrobiology , paleontology , biology
Myasthenia gravis (MG) is an autoimmune disease mediated by circulating autoantibodies (anti‐AchR, anti‐MuSK, etc.). More than 20% of myasthenic patients are refractory to conventional treatments (plasma exchange, IVIg, steroids, azathioprine, mycophenolate mofetil). Rituximab (B‐lymphocyte‐depleting anti‐CD20) and apheresis (double‐filtration plasmapheresis [DFPP] and immunoadsorption [IA]) are interesting therapeutic alternatives. Methods This monocentric pilot study included nine refractory myasthenic patients (March 2018 to May 2020) treated by DFPP and/or IA associated with rituximab (375 mg/m 2 ). Clinical responses were assessed using the Myasthenia Gravis Foundation of America (MGFA) score. Results Average age of patients was 53 ± 17 years. Gender ratio (M/F) was 3:6. The combination of apheresis and rituximab reduced median MGFA score from IV to II after 12 months of follow‐up. Clinical improvement assessed by MGFA score was sustained in the long‐term for all patients, during an average follow‐up of 14 ± 9 months, allowing them to be self‐sufficient and out sick‐leave. The median number of apheresis sessions was 7 (5‐30). The dose of prednisolone was reduced in two patients from 40 mg/d and 30 mg/d to 7.5 mg/d and 10 mg/d, respectively. It was stopped in a patient who was taking 30 mg/d. No infectious, bleeding, or thrombosis complications were noted. Conclusion The combination of rituximab and DFPP was effective to treat refractory MG.