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)

Table 1 Variables rank limited to the 3rd quartile

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

ISSN: 1546-0096