Autoinflammatory disease | Gene mutated | Activator | IL-1 antagonist used | References |
---|---|---|---|---|
Muckle-Wells syndrome (MWS) | NALP3 (CIAS1/NLRP3) | Â | Anakinra | |
 |  |  | Rilonacept | [79] |
Familial cold autoinflammatory syndrome (FCAS, FCU) | NALP3 (CIAS1/NLRP3) | Â | Anakinra | |
 |  |  | Rilonacept | [79] |
Chronic infantile neurological cutaneous and articular syndrome/neonatal onset multisystem inflammatory disease (CINCA/NOMID) [43] | NALP3 (CIAS1/NLRP3) | Â | Anakinra | |
 |  |  | Rilonacept | [79] |
Familial Mediteranean fever (FMF) [44] | MEFV | Â | Anakinra | |
Pyogenic arthritis, pyoderma gangrenosum and acne syndrome (PAPA) | PSTPIP1 | Â | Anakinra | |
Vitiligo [86] | NALP1 (NLRP1) | Â | ? | Â |
Gout | Â | MSU | Anakinra | |
Pseudogout | Â | CPPD | Anakinra | [60] |
Hyperimmunoglobulin D syndrome (HIDS) | Mevalonate kinase | Â | Anakinra | |
Systemic-onset juvenile idiopathic arthritis (SoJIA) | Â | Â | Anakinra | Â |
 |  |  | Rilonacept | [79] |
Behçet's disease (BD) | IL-1β polymorphism |  | Anakinra | |
Schnitzler's syndrome | Â | Â | Anakinra | [77] |
Blau syndrome (BS)/early onset sarcoidosis | NOD2 | Â | Anakinra | [160] |
Crohn's disease (CD) | NOD2 | Â | Anakinra (Ineffective) | [158] |
MEFV (in a proportion of patients) | Â | ? | Â | |
Other diseases | Â | Â | Â | Â |
   Hydatidiform mole [87] | NALP7 (NOD12, NLRP7) |  | ? |  |
   Hypertension [54] | NALP3 |  | ? |  |
   Asthma [175] | NOD1 |  | ? |  |