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From the Department of Neurology (Drs. Schmidt, Harzheim, and Schlegel), Institute of Experimental Hematology and Transfusion Medicine (Dr. Hertfelder), Department of Anesthesiology (Drs. von Spiegel and Hering), Department of Neuropathology (Dr. Deckert-Schlüter), University of Bonn, Germany; and the Neurological Institute (Dr. Lassmann), University of Vienna, Austria.
Address correspondence and reprint requests to Dr. Uwe Schlegel, Department of Neurology, University of Bonn, Sigmund-Freud-Str. 25, D-53105 Bonn, Germany.
Interferon beta-1b (IFNß-1b) is a potent immunomodulatory drug in the treatment of MS. We report a lethal capillary leak syndrome after the administration of IFNß-1b in a patient with disseminated white matter disease, monoclonal gammopathy, and acquired C1 inhibitor (C1-INH) deficiency. IFNß-1b may cause a transient release of proinflammatory cytokines finally resulting in an uninhibited activation of the complement cascade in patients with C1-INH deficiency.
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