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Table 1 Variables rank limited to the 3rd quartile

From: An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA)

Rank Variable Score Frequence Mean score
1 Regular periodicity 436 56 7.8
2 Aphtous stomatitis 431 77 5.6
3 Response to steroid 401 66 6.1
4 Cervical adenitis 368 72 5.1
5 Well-being between flares 299 57 5.2
6 Pharyngitis (exudative or not) 288 47 6.1
7 Increase of acute phase reactants and serum amyloid A during fever episodes 271 44 6.2
8 Normal growth/development 236 51 4.6
9 Pharingotonsillitis 228 35 6.5
10 Periodic fever 3–5 days 202 24 8.4
11 Periodic fever 3–6 days 202 23 8.8
12 Self-limiting episodes 183 35 5.2
13 Response to tonsillectomy 182 33 5.5
14 Improvement with age 160 40 4.0
15 Exclusion cyclic neutropenia/immunodeficiency 150 34 4.4
16 Normalization of acute phase reactants in well-being 146 33 4.4
17 Recurrence every 3–6 weeks 145 21 6.9