Table 4.

Variables falling in the third quartile, considering the total score obtained for cryopyrin-associated periodic syndrome (CAPS).

RankVariableScoreFrequency of CitationMedium Score
1Positive genetic analysis for NLRP3 gene554717.8
2Urticarial rash§ #479667.3
3Response to IL-1 β blockade469696.8
4Recurrent fever§434557.9
5Increase of acute-phase reactants and serum amyloid A during fever episodes #331526.4
6Hearing loss§ #314575.5
7Episodes triggered by cold exposure#291505.8
8Age at onset < 1 yr185286.6
9Chronic urticaria§177296.1
10Chronic meningitis#171394.4
11Increase of acute-phase reactants and serum amyloid A during fever episodes and between episodes166285.9
12Chronic disease course165295.7
13Fever157227.1
14Positive family history151344.4
15Eye involvement§104195.5
16Neurologic involvement#102185.7
17Positive NLRP12 genetic test94146.7
18Conjunctivitis§85204.3
19Osteoarthropathy#74145.3
20Arthralgia#70242.9
21Cartilage overgrowth#66144.7
22Age at onset < 1 mo66116.0
  • § Variable already present in the Eurofever criteria.

  • # Variable already present in the CAPS criteria. IL: interleukin.