Similarly, data derived from the Eurofever Registry and related to 496 patients with FMF, CAPS, TRAPS, MKD, pyogenic arthritis-pyoderma gangrenosum-acne (PAPA) syndrome, deficiency of IL-1 receptor antagonist (DIRA), NLRP12-related autoinflammatory disorder, and periodic fever-aphthosis-pharyngitis-adenitis (PFAPA) syndrome pointed out the key-role of IL-1 blockade for DIRA and CAPS, as well as for cases of poorly controlled MKD, TRAPS, PAPA, and crFMF [72]. Here, IL1B is linked to Pyogenic arthritis - pyoderma gangrenosum - acne.