Zusammenfassung
Die klinische Variabilität des systemischen Lupus erythematodes im Kindes- und Jugendalter
ist ähnlich stark ausgeprägt wie beim Erwachsenen mit einen Trend zu schwereren Verläufen.
So besteht das klinische Spektrum aus einer Spannweite zwischen milden hämatologischen
Auffälligkeiten bis hin zu schweren diffus proliferativen Glomerulonephritiden und
Vaskulitiden. Bei Erkrankungsbeginn weisen bis zu etwa 80 % der betroffenen Kinder
und Jugendliche eine Glomerulonephritis, etwa 60 % eine Hautbeteiligung auf. Die Symptomenkonstellation
und Krankheitsaktivität definiert die Wahl des Therapiekonzeptes, die Intensität und
die Dauer. Sie reichen von immunmodulatorischen über immunsuppressiven bis hin zu
zytotoxischen Medikamenten. Da im Kindes- und Jugendalter keine randomisiert vergleichenden,
prospektiven Studien zur Therapie des SLE existieren, muss für die Therapieentscheidung
auf Studien an erwachsenen Patienten zurückgegriffen werden. Dies erscheint gerechtfertigt,
da sich die Prognose in Abhängigkeit von der Manifestationsart im Kindes- und Jugendalter
nicht wesentlich vom Erwachsenenalter unterscheidet. Die Wahl von remissionsinduzierender
und erhaltender, konventioneller Therapie ist in den letzten 10 Jahren kontrovers
diskutiert und untersucht worden. Diese Wahl wird beeinflusst nicht zuletzt durch
die langfristigen Nebenwirkungen, die von besonderer Bedeutung, gerade im Kindesalter
sind. Die vorliegende Übersichtsarbeit soll helfen, dieses Spannungsfeld aufzuzeigen
und die vorhandenen Ergebnisse von Untersuchungen vom Kindes- bis zum Erwachsenenalter
zusammenfassen.
Abstract
The clinical variability of systemic lupus erythematosus (SLE) in childhood and adolescence
is comparable to that in adulthood, but with a trend to more severe disease courses.
The clinical spectrum ranges form mild haematological disturbances to severe diffuse
proliferative glomerulonephritis and vasculitis. At disease onset about 80 % of affected
children and adolescents present with glomerulonephritis, while 60 % present with
dermatological manifestations. The clinical presentation and the disease activity
define the choice of medication as well as the intensity and duration of therapy.
Immunomodulatory, immunosuppressive and cytotoxic drugs are used in SLE. Since, in
childhood and adolescence populations, there are no prospective and comparative studies
available, the treatment decision in part relies on the experience obtained in adult
patients. Of note, the prognosis with regard to clinical symptoms and disease activity
does not differ in childhood when compared to adulthood. During the last 10 years
the choice of remission-inducing and remission-sustaining conventional therapy has
been debated controversially. This choice is influenced by the rate of side effects,
which are of particular importance in childhood. The current review summarises the
clinical experiences in childhood and adulthood and gives a comprehensive overview
of the available literature.
Schlüsselwörter
systemischer Lupus erythematodes - konventionelle Therapie - Induktionstherapie und
Erhaltungstherapie - Cyclophosphamid - Mycophenolatmofetil
Key words
systemic lupus erythematosus - conventional therapy - children - cyclophosphamide
- mycophenolate mofetil
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Prof. Dr. Hermann J. Girschick
Universitäts-Kinderklinik, Würzburg
Josef-Schneider-Str. 2
97080 Wuerzburg
Phone: ++ 49/9 31/20 12 77 28
Fax: ++ 49/9 31/2 99 67 37
Email: Hermann.Girschick@mail.uni-wuerzburg.de
