Adulter Morbus Still – Neues zur Diagnostik und Therapie

Zusammenfassung

Der adulte Morbus Still (Englisch: adult-onset Still’s disease – AOSD) ist eine seltene auto-inflammatorische Erkrankung bisher unbekannter Ätiologie und Pathogenese. Dabei kann von einem Krankheitskontinuum der systemischen juvenilen idiopathischen Arthritis (sJIA) ausgegangen werden. Die Diagnose wird nach Ausschluss infektiöser, hämatologischer und autoimmuner Erkrankungen gestellt. Hier müssen insbesondere Sepsis, Endokarditis, Neoplasien und Kollagenosen ausgeschlossen werden. Zu den Hauptsymptomen des AOSD zählen u. a. Fieber, Gelenkbeteiligung, Halsschmerzen sowie lachsfarbenes Exanthem. Aufgrund des Fiebers gehört das AOSD zu den Differenzialdiagnosen bei Patienten mit Fieber unklarer Genese und wird in bis zu 3–20% der Fälle diagnostiziert. Zur Klassifikation liegen unterschiedliche Kriterien vor, wobei am häufigsten die nach Yamaguchi (1992) oder Faultrel (2002) eingesetzt werden. Neue Erkenntnisse zur Pathogenese deuten auf eine wichtige Rolle der IL-1 und IL-6 Signalwege hin. Die diagnostische Wertigkeit von neuen Biomarkern wie S100 Proteine und IL-18 muß beim AOSD noch evaluiert werden. Die aktuellen Therapieempfehlungen basieren überwiegend auf Erfahrungswerten, wobei initial NSAR und Glukokortikoide eingesetzt werden. Zu konventionellen und biologischen Basismedikamenten sind bisher keine kontrollierten Studien bei AOSD publiziert. Aus der klinischen Erfahrung und Behandlungsergebnissen des sJIA können verschiedene Therapieansätze für den AOSD extrapoliert werden, dabei zeigen insbesondere eine IL-1 oder IL-6 Blockade gute Effekte. Auf Grundlage des Krankheitskontinuums und neuer molekularbiologischer Daten wurde aktuell eine Erweiterung der Zulassung für den IL-1 Inhibitor Canakinumab von der sJIA auf das AOSD durch die EMA ausgesprochen.

Abstract

Adult-onset Still’s disease (AOSD) is a rare autoinflammatory disease of unknown aetiology and pathogenesis. AOSD is assumed to be a disease continuum of systemic juvenile idiopathic arthritis (sJIA). The diagnosis should be made after ruling out infections, haematologic disorders and autoimmune diseases. In particular, sepsis, endocarditis, neoplasia and connective tissue diseases must be ruled out. The main symptoms of AOSD include fever, joint involvement, sore throat and a salmon-coloured rash. Due to the symptom of fever, AOSD is a differential diagnosis in patients with fever of unknown origin and is diagnosed in up to 3–20% of cases. Yamaguchi’s (1992) and Faultrel’s (2002) criteria are the most commonly used tools for classification. An important role of IL-1 and IL-6 signalling has been suggested based on new insights into the pathogenesis. The diagnostic value of new biomarkers such as S100 proteins or IL-18 has yet to be evaluated. Current treatment recommendations are mainly based on experience, with treatment initially including NSAIDs and corticosteroids. There is no published data from controlled trials with conventional or biologic antirheumatic drugs in AOSD. Clinical experience and treatment results in sJIA lead to different therapeutic approaches for AOSD. In particular, the blockade of IL-1 or IL-6 has demonstrated good effects. Based on an assumed disease continuum and new biomolecular data, the EMA announced that the authorisation for the IL-1 inhibitor canakinumab will be extended to include AOSD in addition to sJIA.

Schlüsselwörter

Adulter Morbus Still - Fieber unklarer Genesé - Arthritis - auto-inflammatorische Erkrankungen

Key words

adult-onset Still’s disease, - fever of unknown origin - arthritis - autoinflammatory diseases

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