Table 2.

Autoinflammatory diseases responsive to interleukin (IL)-1 blockade

Syndrome	Studies
Monogenic disorders other than CAPS and DIRA
Other diseases with IL-1 response
Familial Mediterranean fever (FMF)	[78,119]–[125]
TNF receptor-associated periodic syndrome (TRAPS)	[126]–[129]
Hyper immunoglobulin (Ig)D syndrome (HIDS)	[130,131]
PAPA syndrome†	[132,133]
Polygenic disorders‡
SOJIA§	[71,72,134]
AOSD¶	[135]–[141]
PFAPA	[73]
Behçet's disease	[77]
Schnitzler syndrome	[74]–[76]
‘Metabolic’ autoinflammatory diseases
Gout	[81]–[83]
Diabetes type 2	[84]

^†Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome.

^‡These probable polygenetic diseases do not yet have any genetic mutations or polymorphisms identified but are believed to be caused by genetic predispositions.

^§Only studies with more than 10 patients are listed.

^¶Only studies with more than five patients are listed. DIRA: deficiency of the interleukin (IL)-1 receptor antagonist; CAPS: cryopyrin-associated periodic syndromes; PAPA: pyogenic arthritis, pyoderma gangrenosum and acne syndrome; FPAPA: periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis; SOJIA: systemic onset juvenile idiopathic arthritis; AOSD: adult-onset Still's disease; TNF: tumour necrosis factor.