Overview
Adult-onset Still's disease (AOSD) is a rare inflammatory condition that manifests primarily as arthritis, high fever, and a distinctive salmon-colored rash. Affecting adults, typically between the ages of 20 and 40, it also presents with symptoms like enlarged lymph nodes, spleen, and liver. Though the exact cause remains unclear, AOSD is believed to be an autoimmune disorder. Treatment strategies include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, disease-modifying antirheumatic drugs (DMARDs), and advanced biologics to control inflammation and prevent complications.
Epidemiology of Adult-Onset Still’s Disease
Adult-onset Still’s disease (AOSD) is a rare and often underdiagnosed inflammatory disorder with an estimated global prevalence of 1 in 100,000 individuals. While the condition can affect individuals of any gender or ethnicity, it is notably more common in women and typically develops in adults aged 20 to 40 years.
Geographically, the highest incidence rates are reported in Japan and East Asia, where the condition is better recognized, potentially due to increased awareness and diagnostic advancements. However, AOSD is a global phenomenon, with cases documented in North America, Europe, and other regions, albeit less frequently.
Despite its rarity, the true prevalence may be underestimated due to the overlapping symptoms of AOSD with other inflammatory or autoimmune diseases, which can complicate diagnosis.
Factors and Considerations:
Genetic Susceptibility: Certain genetic markers, including variations in HLA genes, have been linked to a heightened risk, particularly in East Asian populations.
Environmental Triggers: Though not fully understood, infectious agents (such as viral or bacterial infections) are suspected to act as catalysts in genetically predisposed individuals.
Gender and Hormonal Influences: The higher prevalence in women suggests a potential role for hormonal factors in disease development, warranting further research.