Table 1.

Main consensus statements used for the formation of a treatment algorithm for AOSD and level of evidence

	Statement	Agreement	Level of evidence	Grade	References
1	High dose glucocorticoids are effective in AOSD, but due to side effects, the aim should be to limit the frequency and duration of treatment	100%	2B	B	[1, 6, 11–13]
2	IL-1 blockade is an effective treatment early in the AOSD disease course, including in glucocorticoid naïve patients	88%	2B	B	[12, 14–17]
3	In case of life-threatening symptoms of AOSD, treatment with a combination of glucocorticoids with a biologic or glucocorticoid monotherapy should be started as soon as possible	88%	2B	C	[2, 12, 13]
4	If bacterial sepsis is unlikely, starting with short-acting IL-1 blockade in case of life-threatening and rapidly progressing AOSD is justified even if not all test results are known yet	100%	1B	B	[15] and expert opinion
5	In case treatment is started with short-acting IL-1 blockade, the diagnostic process is disrupted less than when starting GC (besides a possible decrease in serum cytokine levels)	100%	4	D	Expert opinion
6	IL-6 blockade is a treatment option in glucocorticoid-resistant or glucocorticoid-dependent AOSD	100%	2B	C	[18–30]
7	IL-1 or IL-6 blockade is preferred in AOSD over TNF blockade; however, TNF blockade may be useful in some patients with persistent arthritis (without systemic manifestations) in the course of their disease	100%	5	D	[18, 21–25, 27–36]
8	Adding MTX may be useful for the treatment of arthritis in AOSD, but there is little evidence for treatment in AOSD with systemic features	94%	4	C	[37–47]

AOSD: adult-onset Still’s disease.