Table 2.

Variables falling in the third quartile, considering the total score obtained for tumor necrosis factor receptor–associated autoinflammatory syndrome (TRAPS).

RankVariableScoreFrequency of CitationMean Score
1Positive genetic analysis for TNFRSF1A gene637748.6
2Increase of acute-phase reactants and serum amyloid A during fever episodes323506.5
3Recurrent prolonged episodes of fever301358.6
4Periorbital edema§286555.2
5Positive family history§282485.9
6Irregular, long-lasting fever episodes254318.2
7Abdominal pain251574.4
8Myalgia§176394.5
9Fever lasting more than 7 days§169237.3
10Localized intense myalgia§135235.9
11Rash§129324.0
12Migratory rash§128274.7
13Duration of attacks 1–3 weeks121158.1
14Arthralgia119323.7
15Monocytic fasciitis114176.7
16Increase of acute-phase reactants and serum amyloid A during fever episodes and between episodes114176.7
17Fever lasting more than 5 days§86108.6
18Conjunctivitis86204.3
19Recurrent episodes of fever82108.2
20Painful maculopapular rash82126.8
  • § Variable already present in preliminary Eurofever criteria.