Abstract
ABSTRACTIntroduction: Adult onset Still’s disease (AOSD) is an uncommon systemic inflammatory disease on the clinical spectrum of autoinflammatory disorders. Its presentation and clinical course may result in several well-differentiated phenotypes: from a systemic and highly symptomatic pattern to a chronic articular pattern. Overproduction of numerous pro-inflammatory cytokines is observed in AOSD. Anakinra (ANK), a human interleukin (IL)-1R antagonist, has recently been approved in the EU for the treatment of AOSD.Areas covered: In this review, we discuss the main studies on the efficacy and safety on ANK for the treatment of AOSD. The vast majority of them are retrospective studies and case series.Expert commentary: Overall, ANK is an effective biologic agent for the treatment of AOSD, especially for the systemic pattern and also for those patients who have life-threatening complications, which frequently occur over the course of the disease. The initial dose usually indicated of ANK in adults is 100 mg/day subcutaneously, although dose reduction can be performed in some cases once the disease is under control. The safety profile of ANK is favorable and similar to that described in other rheumatic diseases. In conclusion, ANK is an effective and safe agent for the treatment of AOSD.
Highlights
Adult-onset Stills disease (AOSD) is a rare systemic inflammatory disease of unknown origin affecting mainly young people with an estimated annual incidence between 0.16 and 0.4/100,000 persons worldwide, independently of ethnic group [1,2,3]
Cases of AOSD associated with malignancies have te been described but this association is not common [8,9]. p AOSD is clinically characterized by daily high spiking fever, evanescent maculopapular e skin rash, arthritis, musculoskeletal symptoms, sore throat and hepatosplenomegaly. c Other common manifestations include pharyngitis, lymphadenopathy, pulmonary Acinfiltrates, pleuritis, pericarditis, myocarditis and abdominal pain [3,10,11]
Serum ferritin levels are increased in AOSD and it may be a good marker of disease activity [3,11]
Summary
Adult-onset Stills disease (AOSD) is a rare systemic inflammatory disease of unknown origin affecting mainly young people with an estimated annual incidence between 0.16 and 0.4/100,000 persons worldwide, independently of ethnic group [1,2,3]. In April 2018, the use of ANK (Kineret®) was approved by the EC for the treatment of Still’s disease including SJIA and AOSD encompassing a t spectrum of indication in adults, adolescents, children and infants aged 8 months and ip older with a body weight ≥10 kg. In these cases, ANK can be administered as cr monotherapy or in combination with NSAIDs and/or DMARDs s (https://www.sobi.com/en/investors/kineretr-anakinra-approved-eu-treatment-stillsu disease).
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