Diagnosis and management of adult onset Still's disease

Abstract

AOSD often poses a diagnostic and therapeutic challenge and clinical guidelines are lacking. The emergence of validated diagnostic criteria, discovery of better serological markers, and the application of new biological agents may all provide the clinician with significant tools for the diagnosis and management of this complex systemic disorder.

Adult-onset Still’s disease

“…These include viruses, such as rubella, CMV, EBV, human herpes virus 6, mumps, echovirus 7, parainfluenza, Coxsackievirus B4, adenovirus, influenza A, HBV, HCV and parvovirus B19, and others microbial agents like Mycoplasma pneumoniae, Chlamydia pneumoniae, Yersinia enterocolitica, Brucella abortus, and Borrelia burgdoferi [1,13].…”

“…An increased sedimentation rate is universal and there is no association with rheumatoid factor (RF) or antinuclear antibody (ANA) [1].…”

“…The therapy of AOSD includes NSAIDs, glucocorticoids, and other disease modifying anti-rheumatic drugs (DMARDs) to control arthritis and systemic disease [1,4].…”

“…Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology, characterised by spiking fever, usually exceeding 39° C, an evanescent salmon-pink rash, arthritis and multiorgan involvement [1,2]. The disorder owes its name to Sir George Frederick Still, who, in 1897, described twenty-two children with symptoms consistent with what is currently known as systemic onset juvenile idiopathic arthritis [3].…”

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

“…It usually occurs in young people between 16 and 35 years of age [1]; women are affected slightly more frequently than men [2][3][4]. The aetiology of AOSD has not been elucidated, but a combination of genetic and infectious factors in the setting of an immune dysregulation has been suggested with an alteration in cytokine production in favour of a Th1 predominance [5].…”