Table 2.

The main cytokines involved in major monogenic autoinflammatory diseases

Defining cytokine
IL‐1β
CAPS (FCAS, MWS, CINCA)
DIRA
HIDS/MKD
IL‐1β and others
TRAPS
FMF
Multiple cytokines via NF‐κB activation
FCAS 2
BS/PGA
CAMPS
INF type I
PRAAS/CANDLE
AGS 1/2/3/4/5/6/7
Deficiency of IL‐10 signalling
EO‐IBD (IL‐10 receptor deficiency, IL‐10 deficiency)
IL‐36
DITRA

CAPS = Cryopyrin‐associated periodic syndrome; FCAS = Familial cold autoinflammatory syndrome; MWS = Muckle–Wells syndrome; CINCA = Chronic infantile neurological, cutaneous and articular syndromes; DIRA = Deficiency of IL‐I receptor antagonist; HIDS = Hyperimmunoglobulinaemia D with periodic fever syndrome; MKD = Mevalonate kinase deficiency; TRAPS = Tumour necrosis factor receptor‐associated periodic syndrome; FMF = Familial Mediterranean fever; FCAS2 = Familial cold autoinflammatory syndrome 2; BS = Blau syndrome; PGA = Paediatric granulomatous arthritis; CAMPS = CARD14‐mediated psoriasis; CANDLE = Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature; PRAAS = Proteasome‐associated autoinflammatory syndrome; AGS = Aicardi‐Goutieres syndrome; EO‐IBD = Early‐onset inflammatory bowel disease; DITRA = Deficiency of IL‐36 receptor antagonist.