Expert consensus on the treatment of patients with adult-onset still's disease with the goal of achieving an early and long-term remission

Giacomelli R.;Caporali R.;Ciccia F.;Colafrancesco S.;Dagna L.;Govoni M.;Iannone F.;Leccese P.;Montecucco C.;Pappagallo G.;Pistone G.;Priori R.;Ruscitti P.;Sfriso P.;Cantarini L.;Belfiore N.;Bernardi C.;Gabini M.;Bettio S.;Brucato A.;Italiano G.;Cantatore F. P.;Iacono D.;Pantano I.;Tirri E.;Ursini F.;Monaco A. L.;Caso F.;Quartuccio L.;Chimenti M. S.;Gattamelata A.;Gremese E.;Paroli M.;Picchianti-Diamanti A.;Sebastiani G. D.;Favalli E.;Sulli A.;Frassi M.;Faggioli P.;Foti R.;Campochiaro C.;Cavalli G.;Tomelleri A.;Manara M.;De Stefano L.;De Angelis R.;Parisi S.;Lopalco G.;Piga M.

Membro del Collaboration Group

;Marotto D.;Colaci M.;Padula A.;Guggino G.;Emmi G.;Baldini C.;Sota J.;Vitale A.;Berti A.;Bartoloni E.;Grava C.;Bindoli S.;Vitetta R.

2023-01-01

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Abstract

We performed a comprehensive systematic targeted literature review and used the Delphi method to formulate expert consensus statements to guide the treatment of adult-onset Still's disease (AOSD) to achieve an early and long-term remission. Seven candidate statements were generated and reached consensus in the first round of voting by the panel of experts. We postulate: (i) In patients with AOSD with predominant arthritis at onset who achieved no disease control with glucocorticoids (GCs), the use of methotrexate can be considered, whereas the use of cyclosporin A and low-dose GCs should not (Statements 1–3); (ii) In patients with AOSD with poor prognostic factors at diagnosis, an IL-1 inhibitor (IL-1i) in addition to GCs should be taken into consideration as early as possible (Statement 4); (iii) A switch to an IL-6 inhibitor (IL-6i) may be considered in patients with AOSD with prevalent joint involvement, who are unresponsive or intolerant to IL-1i (Statement 5); (iv) Drug tapering or discontinuation may be considered in patients who achieved a sustained clinical and laboratory remission with IL-1i (Statement 6); (v) In patients with AOSD who failed to attain a good clinical response with an IL-1i, switching to an IL-6i may be considered in alternative to a different IL-1i. TNF-inhibitors may be considered as a further choice in patients with a prominent joint involvement (Statement 7). These statements will help clinicians in treatment decision making in patients with AOSD.