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Systemischer Lupus erythematodes im Kindes- und Jugendalter

Systemic lupus erythematosus in childhood and adolescence

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Zusammenfassung

Der systemische Lupus erythematodes (SLE) zeichnet sich durch eine Entzündung der Blutgefäße und des Bindegewebes aus, gehört zu den Kollagenosen und gilt als Prototyp der Autoimmunerkrankungen. Er ist eine entzündliche, nicht infektiöse Erkrankung bei Patienten mit genetischer Disposition. Differenzialdiagnostisch muss er gegenüber Malignomen, Infekten und anderen rheumatischen Erkrankungen abgegrenzt werden. Der SLE kann multiple Organmanifestationen zeigen. Als Manifestationen sind neben Allgemeinsymptomen, Hauterscheinungen, Nierenbeteiligung, Arthritiden, eine zerebrale Manifestation sowie eine Beteiligung der Leber und des hämatologischen Systems und des Herzens bekannt. Begleitet wird der SLE häufig von Fieber, Müdigkeit und Gewichtsabnahme. Die Therapie basiert auf einer exakten Diagnostik der Schwere und Ausdehnung der Erkrankung. Die Intensität der Therapie ist gegen ihre Nebenwirkungen abzuwägen.

Abstract

Systemic lupus erythematosus (SLE) is a heterogeneous multisystem autoimmune disease. Differential diagnoses to be considered include malignoma, infectious disease, and other rheumatic diseases. Rashes are a typical clinical feature at the time of diagnosis, followed by polyarthritis and renal pathology. Generalised lymphadenopathy and hepatosplenomegaly are common features, while neurological, pulmonary, and cardiac manifestations are infrequent. Renal involvement with SLE is more frequent in children than adults. Improved understanding of the immunopathogenesis of this disorder over the past two decades now affects therapeutic strategies, with treatment being directed at specific immunological targets. Treatment intensity needs to be balanced against its side effects.

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Authors and Affiliations

  1. Pädiatrische Rheumatologie, Klinik für Pädiatrie I, Department für Kinder- und Jugendheilkunde, Medizinische Universität Innsbruck, Anichstraße 35 , 6020, Innsbruck, Österreich

    J. Brunner, T. Jungraithmayr & L. B. Zimmerhackl

  2. Institut für Pathologie, Medizinische Universität Innsbruck, Innsbruck, Österreich

    C. Sergi

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  1. J. Brunner
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  2. C. Sergi
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  3. T. Jungraithmayr
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  4. L. B. Zimmerhackl
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Correspondence to J. Brunner.

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Gewidmet meinem geschätzten Lehrer Prof. Dr. Dr. F.C. Sitzmann, Homburg.

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Brunner, J., Sergi, C., Jungraithmayr, T. et al. Systemischer Lupus erythematodes im Kindes- und Jugendalter. Monatsschr Kinderheilkd 154, 919–929 (2006). https://doi.org/10.1007/s00112-006-1400-x

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  • DOI: https://doi.org/10.1007/s00112-006-1400-x

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