Adult Still's disease. Case report

Abstract

Adult Still's disease (ASD) is a rare medical condition with rare serious complications; however, its risk factors, prognostic factors, and aspects associated with relapses and steroid refractoriness in individuals with this condition are still not clearly understood. It affects young adults (average age 38 years) 2 AE is characterized by the triad of fever, arthralgias/ arthritis and skin rash; however, its clinical presentation may vary. The most commonly used diagnostic criteria were proposed by Yamaguchi (1992), of which at least 5 are required and 2 of these are major. The major criteria are: fever >39°C lasting ≥1 week, arthralgia or arthritis for at least 2 weeks, flare and leukocytosis >10 000/mm3 with more than 80% polymorphonuclear cells. Minor criteria are odynophagia, recent onset of significant lymphadenopathy,hepatomegaly orsplenomegaly, abnormalliverfunctiontests,and negative tests for antinuclear antibodies (ANA) or rheumatoid factor. Another common finding is hyperferritinemia. Its differential diagnosis is made with infections, malignancy and rheumatic diseases. The prognosis of AAS is favorable, with an estimated specific mortality rate of 1-3%, with Macrophagic Activation Syndrome being its main complication, the prevalence ranges from 10-15% and is associated with a high mortality rate.