Still Disease with Pediatric Onset

Still's disease is an auto-inflammatory disease that occurs in children, and is therefore considered a single nosological entity with clinical and biological similarities. Diagnosis is made on the basis of characteristic cardinal signs, even in the absence of arthritis, which should no longer be an obligatory sign, thus delaying management. Treatment focuses on early, intensive therapy tailored to disease activity, to achieve lasting remission without drugs. Lymphohistiocytic activation syndrome should be a preoccupation of the practitioner throughout the course of the disease.