LAUSR

ABSTRACT Introduction: Adult-onset Still’s disease (AOSD) is an uncommon systemic inflammatory disease characterized by a spiking fever, an evanescent skin rash, polyarthralgia, and hepatosplenomegaly. Although most patients have a benign course, severe manifestations such as macrophage activation syndrome and fulminant hepatitis may occur. These complications could be potentially life-threatening. Therefore, appropriate diagnosis and measurement of disease activity is necessary for the successful treatment of AOSD. Areas covered: Since the disease is very rare, a clinical randomized study cannot be conducted on AOSD. Nevertheless, physicians should be aware of the treatment for AOSD, as the disease can be associated with serious complications and cause mortality. Therefore, here, we aim to provide the appropriate management of patients with AOSD. Expert opinion: Several conventional disease-modifying anti-rheumatic drugs and biologics have been used for patients with AOSD in case series or by observational study. The initial treatment is administration of a corticosteroid for controlling systemic manifestations in AOSD. Methotrexate, cyclosporine, and biologic agents, such as IL-1 antagonists, IL-6 receptor blockers, and TNF blockers, could be considered for treating patients with AOSD refractory to corticosteroids. Ongoing research on the pathogenesis of AOSD can assist in new drug development through the use of new powerful technologies.