Adulter Morbus Still, Schnitzler-Syndrom und autoinflammatorische Syndrome im Erwachsenenalter
作者： P. Lamprecht
DOI: 10.1007/S00393-009-0490-Y
关键词:
摘要: Adult-onset Still's disease (AoSD), Schnitzler syndrome, and cases of adult-onset autoinflammatory syndromes [10-15% familial Mediterranean fever (FMF) tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS)] are characterized by a genetic predisposition, with increased interleukin (IL)-1beta IL-18 production TNF-alpha signaling, respectively. As result, inflammation at barrier tissues (synovial tissues, serous membranes, the skin) encountered in such patients. Pathophysiological insights into these diseases have renewed interest research on IL-1beta rheumatic opened new therapeutic avenues. Recently published studies shown that patients methotrexate-refractory AoSD, colchicine-refractory FMF or contraindications to colchicines respond well treatment soluble IL-1 receptor antagonist anakinra. For TRAPS patients, p75 receptor/Fc-IgG1 fusion protein etanercept is first choice.
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