ABSTRACT

Adult-onset Still's disease is a multisystemic inflammatory disorder, characterised by fever, arthralgia/arthritis, skin rash and neutrophilic leukocytosis. Because it is a rare disease with a heterogenous clinical course, there are no controlled trials concerning different therapeutic options. Treatment decisions are usually based on case reports and retrospective studies involving small patient populations. Non-steroidal anti-inflammatory drugs and corticosteroids are first-line agents. The most frequently used second-line drug in patients refractory to or dependent on steroids is methotrexate. In patients who do not respond to methotrexate, usually biological agents are used. For these patients, firstly anakinra and then anti-TNF agents are recommended.