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IgA-Vaskulitis (Purpura Schönlein-Henoch) bei Kindern und Jugendlichen

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Pädiatrische Rheumatologie

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Zusammenfassung

Die IgA-Vaskulitis (IgAV), früher auch als Purpura Schönlein-Henoch bezeichnet, ist eine leukozytoklastische Vaskulitis mit IgA-haltigen Immunkomplexablagerungen in den kleinen Gefäßen der Haut, des Gastrointestinaltrakts und der Nieren. Klinisch manifestiert sich die Erkrankung mit einer palpablen Purpura, Bauchschmerzen, Nephritis sowie Arthralgien und/oder Arthritis.

Die IgAV ist mit einer Inzidenz von 10–20/100.000 die häufigste Vaskulitis des Kindes- und Jugendalters. Zur klinischen Diagnosestellung sind obligat erforderlich die palpable Purpura sowie mindestens eine weitere Organmanifestation oder der histopathologische Nachweis einer leukozytoklastischen Vaskulitis mit vornehmlich IgA-haltigen Immunkomplex-Ablagerungen.

Die Prognose der IgAV ist sehr gut; in den meisten Fällen ist eine supportive Therapie ausreichend. Die IgAV geht in ca. 30–60 % der Fälle mit einer renalen Beteiligung einher, die in ca. 80 % der Fälle als milde mesangioproliferative Glomerulonephritis mit Hämaturie und/oder geringer Proteinurie verläuft und folgenlos abheilt. In 20 % der Fälle kann es zu einer schweren Nephritis mit nephritischem und/oder nephrotischem Syndrom kommen.

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Authors and Affiliations

  1. Fachbereich Rheumatologie, Klinikum Stuttgart, Pädiatrie 2 (Olgahospital), Stuttgart, Deutschland

    Toni Hospach

  2. Klinik und Poliklinik für Kinder- und Jugendmedizin, Universitätsklinikum Carl Gustav Carus, TU Dresden, Dresden, Deutschland

    Normi Brück

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  1. Toni Hospach
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  2. Normi Brück
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Correspondence to Toni Hospach .

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Editors and Affiliations

  1. Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Aachen, Aachen, Deutschland

    Norbert Wagner

  2. Sektion Rheumatologie der Klinik für Pädiatrie m.S. Pneumologie, Immunologie und Intensivmedizin mit Bereich Rheumatologie, im Sozialpädiatrischen ZentrumCharité - Universitätsmedizin Berlin, Berlin, Deutschland

    Günther Dannecker

  3. Sektion Rheumatologie der Klinik für Pädiatrie m.S. Pneumologie, Immunologie und Intensivmedizin mit Bereich Rheumatologie, im Sozialpädiatrischen ZentrumCharité - Universitätsmedizin Berlin, Berlin, Deutschland

    Tilmann Kallinich

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Hospach, T., Brück, N. (2022). IgA-Vaskulitis (Purpura Schönlein-Henoch) bei Kindern und Jugendlichen. In: Wagner, N., Dannecker, G., Kallinich, T. (eds) Pädiatrische Rheumatologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-60410-6_45

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  • DOI: https://doi.org/10.1007/978-3-662-60410-6_45

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