Treatment of adult-onset still's disease: up to date

Abstract

Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology, and approximately 60-70% of patients may develop a chronic polyphasic form of the disease or a chronic polyarthritis. Due to rarity of disease, treatment of AOSD is not based on controlled study, but on case based experiences. Areas covered: Recently, the application of anti-cytokine therapy based on pathophysiology has resulted in significant progress in the treatment of AOSD. Here, we review current knowledge of the pathogenesis, disease progression, currently available biomarkers of disease activity, standard therapeutic agents, utility of biologic agents, future perspectives for treatment and treatment of macrophage activation syndrome. Expert commentary: Accumulated clinical data suggest that chronic disease can be classified into two subsets: dominant systemic disease, and the arthritis subgroup. IL-1 inhibitors may be more efficient for systemic manifestations and IL-6 inhibitor for both joint involvement and systemic manifestations. TNF inhibitors must be reserved for patients with purely chronic articular manifestations. For ideal management of patients, it is very important to measure disease activity accurately during follow up, but no single biomarker has been classified as ideal. New therapeutic agents and composite biomarkers are needed to improve the outcome of patients with AOSD by identifying disease activity properly.

Keywords: Adult onset Still’s disease; DMARD; anakinra; anti-TNF; biologic; macrophage activation syndrome; methotrexate; tocilizumab; treatment.