Guidelines for the management and treatment of periodic fever syndromes: periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome.
OBJECTIVE
To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.


METHODS
The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation.


RESULTS
806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations.


CONCLUSIONS
1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1β inhibitors, such studies are limited to a few case reports.
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil. Electronic address: teterreri@terra.com.br.
Maria Teresa R A
MT
Terreri
Center for Development of Medical Teaching, Medicine School, Universidade de São Paulo (USP), São Paulo, SP, Brazil.
Wanderley Marques
WM
Bernardo
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil.
Claudio Arnaldo
CA
Len
Pediatric Rheumatology Unit, Children's Institute, Medicine School, Universidade de São Paulo (USP), São Paulo, SP, Brazil.
Clovis Artur Almeida
CA
da Silva
Hospital da Criança de Brasília José Alencar (HCB), Brasília, DF, Brazil.
Cristina Medeiros Ribeiro
CM
de Magalhães
Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brazil.
Silvana B
SB
Sacchetti
Service of Immunology, Allergy and Pediatric Rheumatology, Department of Pediatrics, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (USP), Ribeirão Preto, SP, Brazil.
Virgínia Paes Leme
VP
Ferriani
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil.
Daniela Gerent Petry
DG
Piotto
Service of Rheumatology, Hospital das Clínicas, Universidade Federal de Pernambuco (UFPE), Recife, PE, Brazil.
André de Souza
Ade S
Cavalcanti
Universidade Federal do Pará (UFPA), Belém, PA, Brazil.
Ana Júlia Pantoja
AJ
de Moraes
Service of Rheumatology, Nucleus Adolescents' Health Studies, Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil.
Flavio Roberto
FR
Sztajnbok
Service of Pediatric Rheumatology, Instituto de Puericultura e Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil.
Sheila Knupp Feitosa
SK
de Oliveira
Pediatric Rheumatology Unit, Children's Institute, Medicine School, Universidade de São Paulo (USP), São Paulo, SP, Brazil.
Lucia Maria Arruda
LM
Campos
Hospital Pequeno Príncipe, Curitiba, PR, Brazil.
Marcia
M
Bandeira
Service of Rheumatology, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.
Flávia Patricia Sena Teixeira
FP
Santos
Pediatric Rheumatology Unit, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brazil.
Claudia Saad
CS
Magalhães
D000233:Adenoidectomy; D005334:Fever / Q000175:diagnosis / Q000601:surgery / Q000628:therapy*; D006801:Humans; D008199:Lymphadenitis / Q000175:diagnosis / Q000601:surgery / Q000628:therapy*; D010612:Pharyngitis / Q000175:diagnosis / Q000601:surgery / Q000628:therapy*; D017410:Practice Guidelines as Topic; D013281:Stomatitis, Aphthous / Q000175:diagnosis / Q000601:surgery / Q000628:therapy*; D013577:Syndrome; D014068:Tonsillectomy
Autoinflammatory syndromes; Childhood; Diretrizes; Febre; Fever; Guidelines; Infância; Syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis; Síndrome de febre periódica, estomatite aftosa, faringite e adenite cervical; Síndromes autoinflamatórias