What is the prevalence of Adult-onset Stills Disease?

Adult-onset Still's Disease (AOSD) is a rare systemic inflammatory disorder characterized by high spiking fevers, rash, joint pain, and organ involvement. It was first described in 1971 by George Still and primarily affects adults, although it can also occur in children. The exact prevalence of AOSD is difficult to determine due to its rarity and the lack of comprehensive population-based studies.

However, it is estimated that AOSD affects approximately 0.16 to 0.4 cases per 100,000 individuals worldwide. The disease is more common in females than males, with a female-to-male ratio of around 2:1. AOSD can occur at any age, but the peak incidence is typically between 16 and 35 years old.

The diagnosis of AOSD is challenging as it shares clinical features with other rheumatic diseases. It is often a diagnosis of exclusion, requiring the exclusion of infections, malignancies, and other autoimmune conditions. Treatment usually involves nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and immunosuppressive agents.

While AOSD is a rare condition, it can have a significant impact on the quality of life of affected individuals. Early recognition, accurate diagnosis, and appropriate management are crucial in improving outcomes for patients with AOSD.