Zusammenfassung
Bei dem systemischen Lupus erythmatodes (SLE) handelt es sich um eine Autoimmunerkrankung,
die jedes Organsystem betreffen kann. Die Ätiopathogenese ist bislang nicht vollständig
geklärt. Tiermodelle zeigen, dass B-Lymphozyten bei der Krankheitsentstehung eine
wesentliche Rolle spielen. Therapierefraktäre Verläufe erfordern den Einsatz von Medikamenten,
die für diese spezielle Indikation nicht zugelassen sind, insbesondere betrifft dies
Patienten im Kindesalter. Diese Arbeit gibt einen Überblick über die bislang publizierten
Erfahrungen bei der Anwendung von Rituximab – einem gentechnisch hergestellten monoklonalen
CD-20-Antikörper – bei insgesamt 48 Kindern und Jugendlichen mit SLE. Die Beobachtungszeit
nach erster Anwendung von Rituximab in diesem Kollektiv beträgt bis zu 30 Monate.
Der Therapieerfolg erscheint vielversprechend bei insgesamt guter Verträglichkeit.
Auch wurden Remissionen bei wiederholten Therapiezyklen berichtet. Systematische Anwendungsbeobachtungen
und insbesondere kontrollierte prospektive Studien wären wünschenswert.
Abstract
Systemic lupus erythematosus (SLE) is an autoimmune disorder which can affect every
organ. Its aetiopathogenesis is not fully understood, but animal models have demonstrated
that B lymphocytes play an important role in the pathogenesis of the disease. In refractory
cases, drugs have to be applied which are not approved for this indication, especially
in children. This paper reviews the published data of 48 paediatric patients suffering
from severe SLE who underwent therapy with rituximab – a genetically engineered CD-20
antibody. The total observation period was up to 30 months. Responses seem promising
with a good safety profile altogether. Successful repeated therapies have been reported.
Published data seem to be inhomogeneous giving an evidence level of grade IV. Prospective
studies and especially controlled trials are still needed.
Schlüsselwörter
systemsicher Lupus erythematodes - Rituximab - monoklonale Antikörper - B-Zellen -
Cyclophosphamid
Key words
systemic lupus erythematosus - rituximab - monoclonal antibodies - B cells - cyclophosphamide
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Dr. Annette Jansson
Oberärztin Rheumatologie, Dr. von Haunersches Kinderspital
Lindwurmstr. 4
80337 München
Telefon: ++ 49/89/1 60 78 48
Fax: ++ 49/89/51 60 39 64
eMail: annette.jansson@med.uni-muenchen.de
