Comprehensive Insights into Adult-Onset Still's Disease: Pathophysiology, Diagnosis and Management

Abstract

Adult-onset Still’s Disease (AOSD) is a rare, serious inflammatory disorder characterized by prolonged fevers, and arthritis, with uncommon coloured rash. Although it resembles juvenile idiopathic arthritis, AOSD is diagnosed in patients aged over 16. The disease is fuelled by an overactive immune response, known as a “cytokine storm” and can be set off by infectious agents or genetic factors. It commonly presents as fever, rash, and arthritis, while severe cases may develop rare, but severe, complications, such as Macrophage Activation Syndrome (MAS). The diagnosis of AOSD is made according to certain criteria, including the Yamaguchi criteria, while treatment usually consists of anti-inflammatory drugs, steroids, and other biologics to reduce inflammation and prevent flare-ups. This review will provide a current update on AOSD and will address the pathogenesis, clinical manifestations, diagnosis, and treatment of this disease and the need for early diagnosis with a multidisciplinary approach to treatment.