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Table 2 Publications reporting on the treatment of AOSD with IL-1 inhibitors

From: Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts

StudyDesignPatientsIL-1 treatment
Fitzgerald et al. [57]Case series4 patients with refractory AOSDAnakinra
Kalliolias et al. [58]Case series4 patients with refractory AOSDAnakinra
Kötter et al. [59]Case series4 patients with refractory AOSDAnakinra
Lequerre et al. [60]Multicenter, retrospective observational study35 patients (20 SJIA, 15 AOSD)Anakinra
Franchini et al. [8]Retrospective chart review49 patients with AOSD; 6 treated with biologicsAnakinra (n = 5)
Henderson et al. (abstract) [61]Prospective, open-label, dose escalation study5 patients with AOSDRilonacept
Naumann et al. [62]Case series8 patients with refractory AOSD (3 with systemic disease, 5 with articular disease)Anakinra
Laskari et al. [63]Case series25 patients with refractory AOSDAnakinra
Riera et al. [64]Retrospective review41 patients; 7 treated with biologicsAnakinra (n = 5)
Nordstrom et al. [18]Open, multicenter RCT22 patients with AOSDAnakinra (n = 12) or DMARD (n = 10)
Quartuccio et al. [65]Retrospective, observational study36 patients with AOSDAnakinra (n = 10)
Giampietro et al. [66]Retrospective, observational study28 patients with AOSDAnakinra
Iliou et al. [67]Retrospective observational study44 patients with AOSD; 10 treated with anakinraAnakinra
Gerfaud-Valentin et al. [68]Retrospective observational study57 patients with AOSD; 6 treated with anakinraAnakinra
Hong et al. [69]Meta-analysis8 studies, 134 patients with AOSDAnakinra
Maria et al. [6]Case series8 patients with refractory AOSD treated with biologics; 4 treated with IL-1 inhibitorsAnakinra, canakinumab
Cavalli et al. [70]Retrospective observational study20 patients with AOSD; 16 treated with anakinra, as first biologic option; eventually all patients received anakinraAnakinra
Ortiz-Sanjuán et al. [71]Multicenter, open-label, retrospective study41 patients with AOSD refractory to standard immunosuppressive therapy and to other biologicsAnakinra
Palmou et al. (abstract) [72]Multicenter, open-label study75 patients with AOSD refractory to standard immunosuppressive therapy and to other biologicsTocilizumab (n = 34) or anakinra (n = 41)
Rossi-Semerano et al. [73]Retrospective, observational study189 patients (35 AOSD, 27 SJIA)Anakinra, canakinumab
Lenert and Yao [35]Case series7 patients with AOSD and MASAnakinra (n = 5)
Sfriso et al. [74]Multicenter, retrospective observational study245 patients with AOSD58 patients (23.7%) treated with biologics; anakinra as the first-line biologic (n = 31) or as the second-line biologic (n = 4)
Toz et al. (abstract) [75]Retrospective analysis of database24 patients with refractory AOSD; 7 treated with anakinraAnakinra
Colafrancesco et al. [76]Multicenter retrospective observational study140 patients with refractory AOSD; all treated with anakinra; 4 switched to canakinumab after anakinra failureAnakinra, canakinumab
Junge et al. [77]Comprehensive literature review with data summaries45 articles, > 150 patients with AOSDAnakinra, canakinumab, rilonacept
Feist et al. [33]Subgroup analysis of pooled data from 4 SJIA studies29 older adolescents/younger adults with SJIA, considered representative of patients with AOSDCanakinumab
Neel et al. [78]Multicenter retrospective observational study and systematic literature review20 patients with AOSD admitted to ICU; 5 patients received anakinraAnakinra
  1. AOSD adult-onset Still’s disease, DMARD disease-modifying anti-rheumatic drug, ICU intensive care unit, IL-1 interleukin-1, MAS macrophage activation syndrome, RCT randomized controlled trial, SJIA systemic juvenile idiopathic arthritis