Refractory adult onset Still's disease and hypersensitivity to non-steroidal anti-inflammatory drugs and cyclo-oxygenase-2 inhibitors: are biological agents the solution?

Adult onset Still’s disease (AOSD) is an autoimmune disorder characterised by periodic high fever, arthritis, and typical evanescent rashes. Non-steroidal anti-inflammatory drugs (NSAIDs) are the preferred treatment. In severe cases several disease modifying antirheumatic drugs, thalidomide, and intravenous immunoglobulin have been used. More recently, successful treatment with tumour necrosis factor α (TNFα) blocking agents1,2 and interleukin (IL) 1 neutralisation3 has also been reported. Hypersensitivity to NSAIDs, often characterised by urticaria, angio-oedema, and asthma, has been well documented, and several studies indicate that anaphylactic reactions are related to the inhibition of cyclo-oxygenase-1 (COX-1) enzyme4–6 and that selective COX-2 inhibitors can be safe in these patients. Here we report a case of AOSD complicated by coexisting hypersensitivity …