Raffi Topakian1, Fritz Zimprich2, Stephan Iglseder3, Norbert Embacher4, Michael Guger5, Karl Stieglbauer6, Dieter Langenscheidt7, Jakob Rath2, Stefan Quasthoff8, Philipp Simschitz9, Julia Wanschitz10, David Windisch11, Petra Müller12, Dierk Oel12, Günther Schustereder12, Stefan Einsiedler12, Christian Eggers3, Wolfgang Löscher10. 1. Department of Neurology, Academic Teaching Hospital Wels-Grieskirchen, Grieskirchner Str. 42, 4600, Wels, Austria. raffi.topakian@hotmail.com. 2. Department of Neurology, Medical University of Vienna, Vienna, Austria. 3. Department of Neurology, Krankenhaus Barmherzige Brüder, Linz, Austria. 4. Department of Neurology, University Clinic St. Pölten, St. Pölten, Austria. 5. Clinic for Neurology 2, Med Campus III, Kepler University Clinic, Linz, Austria. 6. Neurologist in Private Practice, Linz, Austria. 7. Department of Neurology, Landeskrankenhaus Rankweil, Rankweil, Austria. 8. Department of Neurology, Graz Medical University, Graz, Austria. 9. Department of Neurology, Klinikum Klagenfurt, Klagenfurt, Austria. 10. Department of Neurology, Medical University Innsbruck, Innsbruck, Austria. 11. Department of Neurology, Landeskrankenhaus Bruck, Bruck, Austria. 12. Department of Neurology, Academic Teaching Hospital Wels-Grieskirchen, Grieskirchner Str. 42, 4600, Wels, Austria.
Abstract
BACKGROUND: Most patients with myasthenia gravis (MG) need long-term immunosuppressive therapy. However, conventional agents may have intolerable side effects, take too long or fail to achieve disease control. Rituximab (RTX) has emerged as an off-label treatment for refractory MG, but data on its use are still sparse. METHODS: We conducted a retrospective nationwide study contacting all Austrian neurologists to provide anonymized data of all adult MG patients treated with RTX and minimum follow-up of 3 months. The Myasthenia Gravis Foundation of America Postintervention Status scale was used to assess outcomes. RESULTS: 34 (60.7%) of a total of 56 patients were women. Median (IQR) age at diagnosis of MG and start of RTX were 41.5 (24.3; 65.8) and 47.5 (33; 71) years, respectively. Antibodies (ab) against acetylcholine receptor (AchR) and muscle-specific tyrosine kinase (MuSK) were present in 69.6% and 25% of patients, respectively (seronegative: 5.4%). Before RTX, 47 (83.9%) patients had had plasma exchange, immune adsorption or immunoglobulins. Three months after RTX, 14 of 53 (26.4%) patients were in remission. At last follow-up after a median of 20 (10; 53) months, remission was present in 42.9% of patients and another 25% had minimal manifestations. Remission was more frequent in patients with MuSK ab vs. those with AchR ab (71.4% vs. 35.9%, p = 0.022). RTX was safe. The presence of MuSK ab independently predicted remission after RTX. CONCLUSION: In this retrospective study on RTX for MG, the largest to date, RTX appeared safe, efficacious and fast acting. Benefit from RTX was greatest in MuSK ab + MG.
BACKGROUND: Most patients with myasthenia gravis (MG) need long-term immunosuppressive therapy. However, conventional agents may have intolerable side effects, take too long or fail to achieve disease control. Rituximab (RTX) has emerged as an off-label treatment for refractory MG, but data on its use are still sparse. METHODS: We conducted a retrospective nationwide study contacting all Austrian neurologists to provide anonymized data of all adult MGpatients treated with RTX and minimum follow-up of 3 months. The Myasthenia Gravis Foundation of America Postintervention Status scale was used to assess outcomes. RESULTS: 34 (60.7%) of a total of 56 patients were women. Median (IQR) age at diagnosis of MG and start of RTX were 41.5 (24.3; 65.8) and 47.5 (33; 71) years, respectively. Antibodies (ab) against acetylcholine receptor (AchR) and muscle-specific tyrosine kinase (MuSK) were present in 69.6% and 25% of patients, respectively (seronegative: 5.4%). Before RTX, 47 (83.9%) patients had had plasma exchange, immune adsorption or immunoglobulins. Three months after RTX, 14 of 53 (26.4%) patients were in remission. At last follow-up after a median of 20 (10; 53) months, remission was present in 42.9% of patients and another 25% had minimal manifestations. Remission was more frequent in patients with MuSK ab vs. those with AchR ab (71.4% vs. 35.9%, p = 0.022). RTX was safe. The presence of MuSK ab independently predicted remission after RTX. CONCLUSION: In this retrospective study on RTX for MG, the largest to date, RTX appeared safe, efficacious and fast acting. Benefit from RTX was greatest in MuSK ab + MG.
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