Adult Onset Still’s Disease (AOSD)

Adult-onset Still’s disease (AOSD), is a systemic, polygenic, auto- inflammatory disorder that is often considered a part of the spectrum of Still’s Disease that includes the better-known systemic juvenile idiopathic arthritis (SJIA), albeit with later age onset. The diagnosis is primarily clinical and necessitates the exclusion of infectious, autoimmune or malignant mimickers. AOSD is a heterogeneous entity, usually presenting with quotidian fevers, arthralgia, skin rash, lymphadenopathy, and hepatosplenomegaly. Complications of AOSD include pulmonary hypertension, macrophage activation syndrome (MAS), diffuse alveolar hemorrhage (DAH), thrombotic thrombocytopenic purpura (TTP) and amyloidosis. Recent advances have revealed a pivotal role of pro-inflammatory cytokines such as interleukin (IL)-1, IL-6, IL-8, and IL-18 in disease pathogenesis, giving rise to the development of novel targeted therapies.