Guidelines for the management and treatment of periodic fever syndromes familial Mediterranean fever.
OBJECTIVE
To establish guidelines based on scientific evidence for the management of familial Mediterranean fever.


METHODS
The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search 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
10,341 articles were retrieved and evaluated by title and abstract; from these, 46 articles were selected to support the recommendations.


CONCLUSIONS
1. The diagnosis of FMF is based on clinical manifestations, characterized by recurrent febrile episodes associated with abdominal pain, chest or arthritis of large joints. 2. FMF is a genetic disease presenting an autosomal recessive trait, caused by mutation in the MEFV gene. 3. Laboratory tests are not specific, demonstrating high serum levels of inflammatory proteins in the acute phase of the disease, but also often showing high levels even between attacks. SAA serum levels may be especially useful in monitoring the effectiveness of treatment. 4. The therapy of choice is colchicine; this drug has proven its effectiveness in preventing acute inflammatory episodes and progression toward amyloidosis in adults. 5. Based on the available information, the use of biological drugs appears to be an alternative for patients with FMF who do not respond or are intolerant to therapy with colchicine.
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
A
de Souza 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
Instituto de Puericultura e Pediatria Martagão Gesteira, Service of Pediatric Rheumatology, 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
D028226:Amyloidosis, Familial / Q000235:genetics / Q000517:prevention & control*; D003078:Colchicine / Q000627:therapeutic use*; D019317:Evidence-Based Medicine; D010505:Familial Mediterranean Fever / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D006801:Humans; D010641:Phenotype; D017410:Practice Guidelines as Topic; D000071198:Pyrin / Q000235:genetics*; D013577:Syndrome
C000604192:MEFV protein, human; D000071198:Pyrin; D003078:Colchicine
Autoinflammatory syndromes; Childhood; Diretrizes; Familial Mediterranean fever; Febre; Febre familiar do Mediterrâneo; Fever; Guidelines; Infância; Síndromes autoinflamatórias