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29.06.2022 | Autoinflammation

Autoinflammation – Unterschiede bei Kindern und Erwachsenen

verfasst von: Martin Krusche, PD Dr. Tilmann Kallinich

Erschienen in: rheuma plus | Ausgabe 4/2022

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Zusammenfassung

Autoinflammatorische Erkrankungen präsentieren sich als multisystemische Entzündungen und werden häufig bereits im frühen Kindesalter manifest. Bei wenigen Erkrankungen, wie z. B. dem kürzlich beschriebenen VEXAS-Syndrom (Vakuolen, E1-Enzym, X‑chromosomal, autoinflammatorisch, somatisch) treten die ersten Krankheitssymptome dagegen ausschließlich im Erwachsenenalter auf. Dieser Beitrag beschreibt, inwiefern sich die phänotypische Ausprägung und Schwere einzelner autoinflammatorischer Erkrankungen in Abhängigkeit vom Lebensalter unterscheiden. Zudem werden altersabhängige Differenzen in der Ausbildung von vorliegenden Organschäden aufgezeigt. Neben den hereditären periodischen Fiebersyndromen werden auch das Krankheitsbild der Adenosindesaminase-2-Defizienz, die Interferonopathien, das PFAPA-Syndrom mit periodischem Fieber, aphthöser Stomatitis, Pharyngitis und zervikaler Adenitis sowie das VEXAS- und das Schnitzler-Syndrom beleuchtet.

Vorheriger Artikel Diagnose und Therapie des nichtspezifischen Kreuzschmerzes

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Titel: Autoinflammation – Unterschiede bei Kindern und Erwachsenen
verfasst von: Martin Krusche
PD Dr. Tilmann Kallinich
Publikationsdatum: 29.06.2022
Verlag: Springer Vienna
Erschienen in: rheuma plus / Ausgabe 4/2022
Print ISSN: 1868-260X
Elektronische ISSN: 2191-2610
DOI: https://doi.org/10.1007/s12688-022-00534-2

Springer Medizin Österreich

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