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Systemische Verlaufsform der juvenilen idiopathischen Arthritis

Systemic onset juvenile idiopathic arthritis

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Zusammenfassung

Die systemische juvenile idiopathische Arthritis (SJIA) ist charakterisiert durch Fieberschübe, ein flüchtiges Exanthem und Arthritis. Die Erkrankung beginnt oft unter dem Bild des Fiebers unklarer Ursache. Bei chronisch aktivem Verlauf droht eine hohe Morbidität: Vor allem Gelenkdestruktionen und ein sekundärer Kleinwuchs sind häufige Komplikationen. Immunologisch steht die Aktivierung des angeborenen Abwehrsystems mit Stimulation der Phagozyten im Vordergrund. Diese wird vermittelt über Interleukin-1 (IL-1), IL-6 und S100-Proteine. Letztere können bei Fieber unklarer Genese differenzialdiagnostisch genutzt werden. Kommt es unter der Behandlung mit Steroiden, Methotrexat und Tumornekrosefaktorblockade zu einem therapieresistenten Verlauf, können neue Therapieansätze mit Antagonisten oder Antikörpern gegen IL-1 und -6 eingesetzt werden. Damit ist zukünftig die Hoffnung einer günstigeren Prognose der SJIA für eine größere Patientengruppe verbunden.

Abstract

Systemic juvenile idiopathic arthritis (SJIA) is characterized by fever, a transient rash and arthritis. The disease often starts with fever of unknown origin. A chronic active course of disease is associated with high morbidity: in particular, joint destruction and secondary growth retardation are common complications. The activation of the innate immune system is the underlying pathogenetic process and is characterized by stimulated phagocytes which secrete interleukin-1 (IL-1), IL-6 and S100 proteins. The latter can be used for differential diagnosis in the situation of fever of unknown origin. In a severe course of SJIA that is resistant to treatment with steroids, methotrexate and TNF blockade, new therapeutic approaches with antagonists or antibodies against IL-1 and IL-6 have been established. Hopefully, these treatment regimens will be associated with a favourable prognosis of SJIA for a larger patient population.

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Authors and Affiliations

  1. Allgemeine Pädiatrie, Klinik und Poliklinik für Kinder- und Jugendmedizin, Universitätsklinikum Münster, Albert-Schweitzer-Campus 1, Gebäude A1, 48149, Münster, Deutschland

    M. Frosch & D. Holzinger

  2. Institut für Immunologie, Universitätsklinikum Münster, Münster, Deutschland

    D. Holzinger & J. Roth

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  1. M. Frosch
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  2. D. Holzinger
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  3. J. Roth
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Correspondence to M. Frosch.

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Frosch, M., Holzinger, D. & Roth, J. Systemische Verlaufsform der juvenilen idiopathischen Arthritis. Monatsschr Kinderheilkd 160, 217–223 (2012). https://doi.org/10.1007/s00112-011-2545-9

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