Table 1.
IL-1 blockade in NLR-related disease
| Autoinflammatory disease | Gene mutated | Activator | IL-1 antagonist used | References |
| Muckle-Wells syndrome (MWS) | NALP3 (CIAS1/NLRP3) | Anakinra | [61-68] | |
| Rilonacept | [79] | |||
| Familial cold autoinflammatory syndrome (FCAS, FCU) | NALP3 (CIAS1/NLRP3) | Anakinra | [65,69-73] | |
| Rilonacept | [79] | |||
| Chronic infantile neurological cutaneous and articular syndrome/neonatal onset multisystem inflammatory disease (CINCA/NOMID) [43] | NALP3 (CIAS1/NLRP3) | Anakinra | [69-71] | |
| Rilonacept | [79] | |||
| Familial Mediteranean fever (FMF) [44] | MEFV | Anakinra | [165-168] | |
| Pyogenic arthritis, pyoderma gangrenosum and acne syndrome (PAPA) | PSTPIP1 | Anakinra | [169,170] | |
| Vitiligo [86] | NALP1 (NLRP1) | ? | ||
| Gout | MSU | Anakinra | [58,59] | |
| Pseudogout | CPPD | Anakinra | [60] | |
| Hyperimmunoglobulin D syndrome (HIDS) | Mevalonate kinase | Anakinra | [166,171] | |
| Systemic-onset juvenile idiopathic arthritis (SoJIA) | Anakinra | |||
| Rilonacept | [79] | |||
| Behçet's disease (BD) | IL-1β polymorphism | Anakinra | [172-174] | |
| Schnitzler's syndrome | Anakinra | [77] | ||
| Blau syndrome (BS)/early onset sarcoidosis | NOD2 | Anakinra | [160] | |
| Crohn's disease (CD) | NOD2 | Anakinra (Ineffective) | [158] | |
| Ulcerative colitis (UC) [52,53] | MEFV (in a proportion of patients) | ? | ||
| Other diseases | ||||
| Hydatidiform mole [87] | NALP7 (NOD12, NLRP7) | ? | ||
| Hypertension [54] | NALP3 | ? | ||
| Asthma [175] | NOD1 | ? |
IL, interleukin; NLR, NOD-like receptor.