Pathogenesis of adult onset still's disease: current understanding and new insights

Abstract

Adult-onset Still's disease (AOSD) is a rare inflammatory disorder typically characterized by fever, arthritis, and hyperferritinemia. Concerning AOSD pathogenesis, it is categorized as a multigenic autoinflammatory disease, at the 'crossroads' of autoinflammatory and autoimmune diseases, because of the involvement of both arms of immune system. Areas covered: This work is conceived as narrative overview assessing the pathogenesis of AOSD. We performed a narrative synthesis of published information, summarizing the contents of previous studies and providing a possible rationale for future researches. MedLine database was searched for identification of suitable studies. In reporting the available evidence, we described the results according to distinct pathogenic steps associated with different clinical features of the disease. Expert commentary: AOSD is a systemic severe inflammatory disorder of unknown etiology affecting young adults. Although pathogenesis of the disease is not fully clarified, the role of the pro-inflammatory cytokines is well-recognized and biologic drugs, blocking these molecules, are routinely used in clinical practice. Finally, given that multiple recent lines of evidence have suggested new insights in AOSD pathogenesis, new therapeutic targets have been highlighted and the results of studies with new drugs could further improve the management of these patients.

Keywords: Adult onset still’s disease; ferritin; inflammation; macrophage activation syndrome; pathogenesis; therapy.