Systemische Juvenile Idiopathische Arthritis – Grenzgänger zwischen Autoinflammation und Autoimmunität

Zusammenfassung

Die Systemische Juvenile Idiopathische Arthritis (SoJIA, Morbus Still) ist ein hochakutes Erkrankungsbild mit einer hohen Rate an Komplikationen, die lebensbedrohlich (Kardiale Beteiligung, Makrophagen-Aktivierungssyndrom) sein können. Mehr als die Hälfte der Patienten haben einen chronischen Verlauf, der nach Abklingen der inflammatorischen Symptome meist als destruierende Polyarthritis verläuft. Ergebnisse der Grundlagenforschung, aber auch die bedeutsamen Erfolge in der Therapie der letzten Jahre zeigen, dass die SoJIA im Akutstadium den autoinflammatorischen Erkrankungen zuordnen ist. Im langfristigen Verlauf zeigt ein großer Teil der Patienten mit einer SoJIA ein chronisch aktives Bild mit Polyarthritis (CAPA-SoJIA) das klinisch, laborchemisch und therapeutisch die Merkmale einer Autoimmunerkrankung aufweist. Die therapeutischen Optionen zur Behandlung der SoJIA haben sich in den vergangenen Jahren, vor allem durch die Einführung der „Biologika” erheblich verbessert, sodass letale Verläufe inzwischen die Ausnahme bilden. Unklar ist bislang, ob es sich bei der SoJIA um eine Entität – einen Grenzgänger zwischen Autoinflammation und Autoimmunität handelt, oder ob die gültigen Klassifikationskriterien mindestens 2 Erkrankungen umfassen.

Abstract

Systemic onset juvenile idiopathic arthritis (SoJIA, morbus Still) is a hyperacute disease associated with a high rate of complications, some of them life-threatening (carditis, macrophage-activating syndrome). More than half of the patients will suffer from a chronic course, mostly accompanied by destructive arthritis, after the symptoms of recurrent systemic inflammation have declined. Data from basic science as well as the significant progress in treatment options point out that SoJIA is an autoinflammatory disease, at least within the acute state of the disease. In the long-term course most of the patients with SoJIA will develop the clinical picture of chronic active disease with polyarthritis (CAPA-SoJIA). In contrast to the acute phase of disease in SoJIA, CAPA-SoJIA shows all clinical and laboratory chemical signs of an autoimmune disease. A change of treatment from antiautoinflammatory to antiautoimmune medications is frequently necessary. Therapeutic options in SoJIA have been substantially improved during the past few years. This is mainly due to the merit of biologics. Fortunately, fatal outcome has become a rare event. It remains unclear whether SoJIA encompasses just one entity – being at the borderline between autoinflammation and autoimmunity, or whether this definition includes more than one disease.

Schlüsselwörter

Systemisch Juvenile Idiopathische Arthritis - Autoimmunität - Autoinflammation - SoJIA

Key words

systemic onset juvenile idiopathic arthritis - autoimmunity - autoinflammation - SoJIA

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