Table 2.
Summary of the evidence.
| disease Still's Adult-onset | disease Behcet's | CAPS / MWS | DIRA | FMF | Gout | GvHD | suppurativa Hidradenitis | HIDS | MAS | arthritis Psoriasis | gangrenosum Pyoderma | pericarditis Recurrent | arthritis Rheumatoid | SAPHO | syndrome Schnitzler's | syndrome Sjögren's | sJIA | TRAPS | T1DM | vasculitis Urticarial | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Biologics | Diseases | |||||||||||||||||||||
| Anakinra | IV | IV | I | – | I | IIa | IIb | IIb | IIIb | IIb | IV | IV | IIb | I | IV | IV | IIb | I | IV | IIb | – | |
| Bermekimab | – | – | – | – | – | – | – | IIIb | – | – | – | – | – | – | – | – | – | – | – | – | – | |
| Canakinumab | IIb | IV | I | – | I | I | – | – | I | – | – | IV | – | IIb | – | IIb | – | I | I | IIb | IV | |
| Gevokizumab | – | IIb | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | IIb | – | |
| Rilonacept | IV | – | I | IV | IIb | IIb | – | – | – | – | – | – | IIb | – | – | IV | – | IIb | – | IIb | – | |
| Legend: | ||||||||||||||||||||||
| Level I | Approved by the EMA and/or FDA | |||||||||||||||||||||
| Level IIa | Multicentric double-blind RCT proving a significant superiority over standard-of-care treatment | |||||||||||||||||||||
| Level IIb | Multicentric double-blind RCT proving a significant superiority over placebo | |||||||||||||||||||||
| Level IIIa | Clinical study, not fulfilling the above-mentioned criteria, but proving a superiority over standard-of-care treatment | |||||||||||||||||||||
| Level IIIb | Clinical study, not fulfilling the above-mentioned criteria, but proving a superiority over placebo | |||||||||||||||||||||
| Level IV | Case series or open-label trials without control group with positive results | |||||||||||||||||||||
| – | No or too little information available | |||||||||||||||||||||
| Achieved | Failed | Mixed results | ||||||||||||||||||||
CAPS, cryopyrin-associated periodic syndromes; DIRA, deficiency in IL-1 receptor antagonist; EMA, European Medicines Agency; FDA, U.S. Food and Drug Administration; FMF, familial Mediterranean fever; GvHD, graft-versus-host disease; HIDS, hyper-IgD syndrome; MAS, macrophage activation syndrome; MWS, Muckle-Wells syndrome; SAPHO, synovitis, acne, pustulosis, hyperhidrosis, osteitis; sJIA, systemic juvenile idiopathic arthritis; TRAPS, TNF receptor-associated periodic syndrome; T1DM, type 1 diabetes mellitus.