Adult-onset Still's disease, Schnitzler syndrome, and autoinflammatory syndromes in adulthood

Adult-onset Still's disease (AoSD), Schnitzler syndrome, and cases of adult-onset autoinflammatory syndromes [10-15% of cases of familial Mediterranean fever (FMF) and tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS)] are characterized by a genetic predisposition, with increased interleukin (IL)-1 beta and IL-18 production and TNF-alpha signaling, respectively. As a result, periodic fever and inflammation at barrier tissues (synovial tissues, serous membranes, and the skin) are encountered in such patients. Pathophysiological insights into these diseases have renewed interest in research on IL-1 beta in rheumatic diseases and have opened new therapeutic avenues. 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.