Table 3 Statements and results of Delphi survey
Statement | Agreement (%) | Level of evidence (range) | Grade of recommendation |
|---|---|---|---|
1.1 Adult Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (SJIA) show substantial similarities in terms of clinical manifestations, laboratory features, response to treatment and, possibly, genetic background. | 94 | 3b–4 | C |
1.2 Adult onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (SJIA) may be considered the same disease. Differences in the rate of clinical manifestations could be related to the age of onset. | 88 | 5 | D |
2.1 There is evidence that IL-1 inhibition represents an effective therapeutic approach in AOSD refractory to conventional treatment and/or other biologics. | 96 | 2a | B |
2.2 In AOSD, treatment with IL-1 inhibitors is effective on different clinical and laboratory parameters and displays a significant steroid sparing effect in most patients. Therapeutic response is rapid and sustained over time. | 100 | 2a | B |
2.3 IL-1 inhibitors are effective in the treatment of AOSD-related Macrophage Activation Syndrome (MAS), although cases of MAS occurring during treatment with these drugs are reported. | 98 | 2b–4 | C |
2.4 IL-1 inhibitors have an overall satisfactory safety profile in AOSD. Among adverse events, infections have been reported. Treatment with anakinra has been associated with frequent injection site reactions and occasionally severe cases of hepatotoxicity, reversible after treatment withdrawal. | 98 | 2b–5 | C |
3.1 A different profile of efficacy among IL-1 inhibitors cannot be established, because head-to-head comparisons are lacking and available data deal with the use of canakinumab and rilonacept almost exclusively after anakinra failure. | 100 | 5 | D |
3.2 Considering available data on efficacy, failure of the first IL-1 inhibitor does not preclude the achievement of a therapeutic response with another IL-1 inhibitor. | 88 | 5 | D |
4.1 There are no available studies comparing the efficacy of the treatment with IL-1 inhibitors in early versus late stages of AOSD. Data from SJIA suggest that early treatment with IL-1 inhibitors is associated with a better therapeutic response. | 96 | 5 | D |
4.2 Indirect data show that IL-1 inhibitors can be effective in AOSD both in first and subsequent lines of biologic treatment. | 98 | 2b | B |
5.1 Some data suggest that IL-1 inhibitors may be more effective on systemic rather that chronic articular manifestations of AOSD. | 98 | 2b | C |