Abstract

Adult onset Still’s disease (AOSD) is a rare clinical entity with unknown etiology, characterized by arthritis, fever, evanescent rash, and other systemic presentations. AOSD generally does not overlap with other rheumatic diseases. AOSD is a diagnosis by exclusion, and it is one of the common causes of pyrexia of unknown origin (PUO) which can be life-threatening if mistreated. Although steroids are the first line of therapy, about 20%-30% of patients are refractory, intolerant, and or relapse during tapering or upon discontinuation of steroids. There are no clinical guidelines in treating such patients, which is challenging.
 Keywords:  Still’s disease, arthritis, fever, auto-inflammatory

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