[Still's disease as biphasic disorder : Current knowledge on pathogenesis and novel treatment approaches]

Still's disease covers a range of disorders from systemic juvenile idiopathic arthritis (SJIA) up to adult onset Still's disease (AOSD). The overlapping clinical features suggest that SJIA and AOSD are different manifestations of a phenotypic continuum in different age stages. Still's disease is clinically characterized by fever, rash, joint involvement, lymphadenopathy and serositis. In this review the more recent pathogenetic model of a biphasic disease course is presented. The initial autoinflammation with predominant dysregulation of innate immunity is the basis of the "window of opportunity" hypothesis for the early use of a cytokine blockade. If the disease is not stopped in this phase, a phenotype change to a disease with destructive arthritis regularly occurs, in which dysregulation of the mechanisms of adaptive immunity plays a special role. The understanding of Still's disease as a biphasic disease enables the monitoring of molecular signatures. At the same time, this opens up perspectives for phase-specific targeted treatment using modern treat-to-target strategies.