Management of AOSD
NSAIDs are used as antipyretics and as analgesics for arthritic pain. The mainstay of treatment is immunosuppression, first-line being oral corticosteroids and then intravenous methylprednisolone in severe cases. If AOSD is refractory, then DMARDs are initiated, inducing remission in around 70% of cases. Methotrexate, azathioprine, hydroxychloroquine, ciclosporin and tacrolimus are used as corticosteroid-sparing agents. There is evidence for the use of IVIG in refractory cases, during pregnancy and in life-threatening secondary HLH. Biologics such as anakinra (an anti-IL-1𝛃 drug) and tocilizumab (an anti-IL-6 drug) can also be used, though anti-TNF-ɑ drugs such as adalimumab and etanercept have reportedly led to secondary HLH and paradoxical AOSD flares in case reports. The choice of initial agent and, indeed, the decision to escalate agents will depend on disease severity and the degree of organ involvement at that time.
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