Classic autoinflammatory diseases |
Familial Mediterranean fever (FMF) |
Pyogenic arthritis, pyoderma gangrenosum, acne (PAPA)*† |
Cryopyrin-associated periodic syndromes (CAPS) |
Hyper IgD syndrome (HIDS) |
Adult and juvenile Still disease |
Schnitzler syndrome |
TNF receptor-associated periodic syndrome (TRAPS) |
Blau syndrome; Sweet syndrome |
Deficiency in IL-1 receptor antagonist (DIRA) |
Probable autoinflammatory diseases |
Recurrent idiopathic pericarditis |
Macrophage activation syndrome (MAS) |
Urticarial vasculitis |
Antisynthetase syndrome |
Relapsing chondritis |
Behçet disease |
Erdheim-Chester syndrome (histiocytosis) |
Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) |
Common diseases mediated by IL-1β |
Rheumatoid arthritis‡ |
Periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome (PFAPA) |
Urate crystal arthritis (gout) |
Type 2 diabetes |
Smoldering multiple myeloma |
Postmyocardial infarction heart failure |
Osteoarthritis |
Classic autoinflammatory diseases |
Familial Mediterranean fever (FMF) |
Pyogenic arthritis, pyoderma gangrenosum, acne (PAPA)*† |
Cryopyrin-associated periodic syndromes (CAPS) |
Hyper IgD syndrome (HIDS) |
Adult and juvenile Still disease |
Schnitzler syndrome |
TNF receptor-associated periodic syndrome (TRAPS) |
Blau syndrome; Sweet syndrome |
Deficiency in IL-1 receptor antagonist (DIRA) |
Probable autoinflammatory diseases |
Recurrent idiopathic pericarditis |
Macrophage activation syndrome (MAS) |
Urticarial vasculitis |
Antisynthetase syndrome |
Relapsing chondritis |
Behçet disease |
Erdheim-Chester syndrome (histiocytosis) |
Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) |
Common diseases mediated by IL-1β |
Rheumatoid arthritis‡ |
Periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome (PFAPA) |
Urate crystal arthritis (gout) |
Type 2 diabetes |
Smoldering multiple myeloma |
Postmyocardial infarction heart failure |
Osteoarthritis |
Complete list of citations is in supplemental References (available on the Blood Web site; see the Supplemental Materials link at the top of the online article).
Incomplete responses to IL-1β blockade have been reported.
The combination of IL-1 plus TNFα have been used in some disorders.
Rheumatoid arthritis is also treatable with other anticytokines, antireceptors, immunomodulating agents, and B cell–depleting antibodies.