《Adult-onset Still disease》.pdf

Best Practice Research Clinical Rheumatology Vol. 22, No. 5, pp. 773–792, 2008 doi:10.1016/j.berh.2008.08.006 available online at 1 Adult-onset Still disease Bruno Fautrel* MD, PhD Professor ´ ˆ ` Pierre Marie Curie – Paris VI University/Paris Universitas, Department of Rheumatology, Pitie-Salpetriere Hospital, ˆ 83 boulevard de l’Hopital, 75651 Paris, Cedex 13, France Adult-onset Still disease (AOSD) is an uncommon inflammatory condition of unknown origin typically characterized by four main (cardinal) symptoms: spiking fever 39 C, arthralgia or ar- 3) with neutrophils 80%. As many thritis, skin rash and hyperleucocytosis ( 10,000 cells/mm other manifestations are possible, diagnosis is potentially challenging. Determination of the total and glycosylated ferritin levels, although not pathognomonic, can help in diagnosis. The disease evolution of AOSD can be monocyclic, polycyclic or chronic. In chronic disease, joint involve- ment is often predominant and erosions are noted in one-third of patients. No prognostic fac- tors have been identified to date. Therapeutic strategies are from observational data. Corticosteroids are usually the first-line treatment. With inadequate response to corticoste- roids, methotrexate appears the best choice to control disease activity and allow for tapering of steroid use. For refractory disease, biological therapy with agents blocking interleukin-1 (ana- kinra) and then those blocking interleukin-6 (tocilizumab) seem the most promising. Key words: anakinra; autoinflammatory syndrome; biologics; ferritin; glycosylated ferriti