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Editorial
. 2017 Jan 6;15(1):5.
doi: 10.1186/s12916-016-0769-1.

About the complexity of adult onset Still's disease… and advances still required for its management

Philippe Guilpain  1   2   3 , Alain Le Quellec  4   5
Affiliations
Editorial

About the complexity of adult onset Still's disease… and advances still required for its management

Philippe Guilpain et al. BMC Med. .

Abstract

Adult onset Still's disease (AOSD) is a rare inflammatory disorder that remains poorly understood. Its pathophysiology is yet to be completely elucidated, but is known to consist mainly on a cytokine cascade, responsible for the systemic manifestations. AOSD diagnosis is usually difficult and delayed, with physicians having to rule out several other conditions, including cancer or infectious diseases. Prognosis is heterogeneous and difficult to establish, ranging from benign outcome to chronic destructive polyarthritis and/or life-threatening events. In addition, treatment remains to be codified, especially considering the development of new drugs. In this commentary, we attempt to elucidate the complexity of AOSD and to highlight the need of working on prognostic tools for this disorder. We also discuss the numerous advances that would be useful for patients in the daily management of this disease.Please see related article: http://bmcmedicine.biomedcentral.com/articles/ 10.1186/s12916-016-0738-8 .

Keywords: Adult onset Still’s disease; Cytokine; Diagnosis; Inflammasome; Macrophage activation syndrome; Prognosis; Score; Therapy.

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References

    1. Maria ATJ, Le Quellec A, Jorgensen C, Touitou I, Rivière S, Guilpain P. Adult onset Still’s disease (AOSD) in the era of biologic therapies: dichotomous view for cytokine and clinical expressions. Autoimmun Rev. 2014;13:1149–59. doi: 10.1016/j.autrev.2014.08.032. - DOI - PubMed
    1. Ruscitti P, Cipriani P, Masedu F, Iacono D, Ciccia F, Liakouli V, Guggino G, Carubbi F, Berardicurti O, Di Benedetto P, Valenti M, Triolo G, Valentini G, Giacomelli R. Adult onset Still’s disease: evaluation of prognostic tools and validation of the systemic score, by analysis of 100 cases from three centers. BMC Med. 2016;14:194. doi: 10.1186/s12916-016-0738-8. - DOI - PMC - PubMed
    1. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19:424–30. - PubMed
    1. Fautrel B, Zing E, Golmard J-L, Le Moel G, Bissery A, Rioux C, et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore) 2002;81:194–200. doi: 10.1097/00005792-200205000-00003. - DOI - PubMed
    1. Pouchot J, Sampalis JS, Beaudet F, Carette S, Décary F, Salusinsky-Sternbach M, et al. Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 1991;70:118–36. doi: 10.1097/00005792-199103000-00004. - DOI - PubMed

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