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Long-term effects of a multimodal approach including immunoadsorption for the treatment of myasthenic crisis
Authors:Zeitler Heike  Ulrich-Merzenich Gudrun  Hoffmann Lars  Kornblum Cornelia  Schmidt Stephan  Vetter Hans  Walger Peter
Affiliation:Medical Policlinic, University of Bonn, Bonn, Germany. heike.zeitler@uni-bonn.de
Abstract:A multimodal treatment protocol with immunoadsorption (IA) as the central element was used in the treatment of myasthenic crisis (MC). Fifteen patients with MC were treated in repeated, uninterrupted 7-day cycles until mobilization with: (i) large-volume IA using an antihuman-IgG adsorber, days 1-5; (ii) intravenous immunoglobulin substitution (0.3-0.5 g/kg body weight [BW]/day), days 5-7; and (iii) immunosuppression with cyclophosphamide (1-2 mg/kg BW/day) and prednisolone (0.5-1 mg/kg BW/day), until remission. Patients required a median of 8 days of mechanical ventilation, 12 days in the intensive care unit, and 35 days of hospitalization. Functional improvement compared to their precrisis condition was attained by 14 of 15 patients. MG severity score improved by a mean of 10 points, quality of life score by 9.8 points, and Karnofsky index by 29 points in 14 of 15 patients. Improvements remained stable and no further crises occurred during long-term follow-up, which averaged 4.4 years. No fatalities due to MC occurred. The results demonstrate that our protocol is a potent therapeutic approach in the treatment of MC.
Keywords:Myasthenic crisis    Immunoadsorption    Immunomodulation    Myasthenia gravis
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