Fig. 1

Cryopyrin associated periodic syndrome disease activity score pre-and post canakinumab. The difference between CAPS DAS scores before and after canakinumab treatment was highly significant (P < 0.0001). A standardised approach was used to monitor CAPS disease activity, as described previously in this clinic [3]. In brief, this consisted of standardised documentation of clinical disease activity using the CAPS disease activity score (DAS) (please see Additional file 1). The DAS depicts disease activity using ten symptoms/signs; absence of disease activity was defined as a score of 0/20; minimal disease activity was depicted by a score ≤ 3/20; and a maximum score of 20 indicated maximal disease activity. The figure shows that before commencing canakinumab treatment, the median CAPS DAS was 6.5/20 (range 0–11/20), with 95% of patients scoring > 3/20 (Fig. 1)