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Adult-onset Still’s disease, Schnitzler syndrome, and autoinflammatory syndromes in adulthood
Zeitschrift für Rheumatologie, 10/29/09

Lamprecht P – Adult–onset Still’s disease (AoSD), Schnitzler syndrome, and cases of adult–onset autoinflammatory syndromes are characterized by a genetic predisposition, with increased interleukin (IL)–1beta and IL–18 production and TNF–alpha signaling, respectively. Recently published studies have shown that patients with Schnitzler syndrome, methotrexate–refractory AoSD, and colchicine–refractory FMF or contraindications to colchicines in FMF respond well to treatment with the soluble IL–1 receptor antagonist anakinra. For TRAPS patients, the p75 TNF–alpha receptor/Fc–IgG1 fusion protein etanercept is the treatment of first choice.

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