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Table 1 IL-1 blockade in NLR-related disease

From: NOD-like receptors and inflammation

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

 

?

 
  1. IL, interleukin; NLR, NOD-like receptor.