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Erschienen in: Clinical Rheumatology 1/2011

01.01.2011 | Original Article

B cell depletion for autoimmune diseases in paediatric patients

verfasst von: Annette F. Jansson, Claudia Sengler, Jasmin Kuemmerle-Deschner, Bernd Gruhn, A. Birgitta Kranz, Hartwig Lehmann, Daniela Kleinert, Lars Pape, Hermann J. Girschick, Ivan Foeldvari, Dieter Haffner, Johannes P. Haas, Dagmar Moebius, Dirk Foell, Joachim Peitz, Veit Grote

Erschienen in: Clinical Rheumatology | Ausgabe 1/2011

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Abstract

Data on B cell depletion therapy in severe autoimmune diseases in paediatric patients are very limited. We conducted a retrospective cohort study and recruited patients who were treated with rituximab (RTX) and followed up for at least 6 months through the German societies of paediatric rheumatology and nephrology. The aim was to describe the spectrum of autoimmune disorders for which RTX was used and to describe the applied therapeutic regimens, the observed efficacy, as well as potential immunological side effects. The need to develop standard treatment guidelines for future trials should be discussed. Sixty-five patients were included. Nineteen patients suffered from systemic lupus erythematosus, 13 from vasculitic disorders, 12 from hematological autoimmune diseases, 5 from mixed connective tissue disorders, 4 from juvenile idiopathic arthritis, and 9 had other autoimmune diseases. Adverse, infusion-related events were reported in 12/65 (18%) patients. Considering laboratory and clinical parameters, 13 patients (22%) were in complete remission, 31 (52%) were in partial remission, 6 (10%) were unchanged and 10 (17%) had progressed after 6 months. In 46% of the patients, the steroid dose could be more than halved. IgG, IgM and IgA decreased from normal levels prior to RTX therapy to below normal levels at 6 months in 2/22 (9%), 10/21 (48%), and 4/22 (18%) patients, respectively. Immunoglobulin deficiency or prolonged CD20 depletion was reported in eight patients after an observation period longer than 12 months. RTX therapy led to a perceivable reduction in disease activity. However, long-term immunological alterations may occur in more than 10% of the patients. Guidelines and protocols for off-label therapy are desirable to document reasonable follow-up data. Controlled prospective studies for RTX therapies in children with standardised therapeutic and diagnostic protocols are urgently needed.
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Metadaten
Titel
B cell depletion for autoimmune diseases in paediatric patients
verfasst von
Annette F. Jansson
Claudia Sengler
Jasmin Kuemmerle-Deschner
Bernd Gruhn
A. Birgitta Kranz
Hartwig Lehmann
Daniela Kleinert
Lars Pape
Hermann J. Girschick
Ivan Foeldvari
Dieter Haffner
Johannes P. Haas
Dagmar Moebius
Dirk Foell
Joachim Peitz
Veit Grote
Publikationsdatum
01.01.2011
Verlag
Springer-Verlag
Erschienen in
Clinical Rheumatology / Ausgabe 1/2011
Print ISSN: 0770-3198
Elektronische ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-010-1630-0

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