Pathogenese des systemischen Lupus erythematodes (SLE) im Kindesalter

Zusammenfassung

Der systemische Lupus erythematodes (SLE) ist eine systemische Autoimmunerkrankung, die jedes Organsystem betreffen kann und deren Erkrankungsgipfel im Erwachsenenalter liegt. Die deutliche Bevorzugung des weiblichen Geschlechts (9–10 : 1) deutet auf hormonelle Einflüsse in der Pathogenese hin. Obwohl seltener als Erwachsene, erkranken auch Kinder und Jugendliche am SLE. Innerhalb der pädiatrischen Altersgruppe fällt eine variable Geschlechterverteilung mit ausgeglichenem Verhältnis vor der Pubertät auf. Danach kommt es zu einer Annäherung an die Erwachsenenverteilung. Die besondere Bedeutung des pädiatrischen SLE liegt im höheren Schweregrad kindlicher Manifestationen mit gesteigerter chronischer Krankheitsaktivität und schlechterer Prognose. Eine Beeinträchtigung der körperlichen Entwicklung kann zum einen aus der chronischen Entzündung und den daraus resultierenden Organschäden, aber auch aus der teils toxischen Therapie resultieren. Die variable Geschlechterverteilung zusammen mit schwereren Verläufen bei jüngeren Patienten wirft die Frage nach unterschiedlichen Pathomechanismen in den verschiedenen Altersgruppen auf. In der vorliegenden Arbeit diskutieren wir anhand der Literatur genetische, hormonelle und Umwelteinflüsse auf die Pathogenese des SLE im Kindes-$$$ und Jugendalter und formulieren ein hypothetisches Modell zur Pathogenese des SLE in den verschiedenen Altersgruppen.

Summary

Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder that can affect any organ of the human body. A peak in disease onset during early adulthood and the female predominance (9–10 : 1) indicate the role of hormonal factors in the pathogenesis of SLE. Though less common when compared to the adult age group, also children can develop SLE. Gender distribution varies in the pediatric age group with equal numbers in the first decade and female predominance thereafter (second decade 4 : 1, then 9–10 : 1). Furthermore, the clinical course in children is more severe with increased chronic disease activity and resulting organ damage. Secondary to these variables in the pediatric age group, the question of whether differential pathomechanisms may be involved in pediatric SLE has been raised. Here, we discuss the contribution of genetic, hormonal and environmental factors in the pathogenesis of SLE in the various age groups.

Schlüsselwörter

Systemischer Lupus erythematodes - SLE im Kindesalter - Pathogenese des SLE

Keywords

Systemic lupus erythematosus - pediatric SLE - pathogenesis of pediatric SLE

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