Juvenile idiopathische Arthritis

Zusammenfassung

Die juvenile idiopathische Arthritis (JIA) ist eine heterogene Erkrankung, die bei Kindern und Jugendlichen auftritt und zur dauerhaften Beeinträchtigung mit großem Leidensdruck führen kann. Bisher ist über die Ätiologie und Pathogenese der JIA noch wenig bekannt und die Behandlung stellt sich schwierig dar, da sich die JIA erst im Verlauf in die verschiedenen Krankheitsformen einordnen lässt. Neben der oligoartikulären JIA gibt es Formen mit polyartikulärer Ausprägung oder systemischen Manifestationen bis hin zum Krankheitsbild mit Makrophagenaktivierungssyndrom. Dieser Artikel wird sich neben der systemischen JIA mit polygenem Erscheinungsbild auch mit den nichtsystemischen Formen der JIA mit monogenem Charakter beschäftigen und den aktuellen Kenntnisstand hinsichtlich Biomarker darstellen. Bei der JIA fehlen, genau wie bei der rheumatoiden Arthritis oder anderen Autoimmunerkrankungen, bis heute valide und kommerziell nutzbare Biomarker für die Diagnose mit schneller Subklassifizierung der Patienten, zur Prognose und Vorhersage des Ansprechens auf die Therapie, um die angestrebte patientenorientierte „individualisierte Medizin“ zu gewährleisten.

Summary

Juvenile idiopathic arthritis (JIA) is a heterogenous chronic rheumatic disease which affects children and adolescents. The etiology and pathogenesis of JIA are still unkown and the treatment is complex. In general, burden of the disease increases with the number of affected joints and with severe systemic inflammation. Beside oligoarticular and polyarticular JIA with monogenetic characteristics, the systemic autoinflammatory form of JIA (sJIA) is characterised by polygenetic involvements. Furthermore, in oligo- or polyarticular JIA regulatory and effector T-cell subsets are affected, whereas in sJIA cells of the myeloid system are involved. So far, not only in rheumatoid arthritis and other autoimmune diseases but also in JIA valuable biomarkers are rare and commercial tests are still missing to diagnose JIA subgroups, to allow prognosis of the disease, and to predict individual therapeutic response. Therefore, validated biomarkers are of need to enhance bench-to-bedside research and development in childhood arthritis.

Schlüsselwörter

Biomarker - juvenile idiopathische Arthritis - Makrophagenaktivierungssyndrom

Keywords

Biomarker - juvenile idiopathic arthritis - macrophage activation syndrome

• Literatur

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