Autor(es):
Terreri, Maria Teresa R.A. ; Bernardo, Wanderley Marques ; Len, Claudio Arnaldo ; da Silva, Clovis Artur Almeida ; de Magalhães, Cristina Medeiros Ribeiro ; Sacchetti, Silvana B. ; Ferriani, Virgínia Paes Leme ; Piotto, Daniela Gerent Petry ; Cavalcanti, André de Souza ; de Moraes, Ana Júlia Pantoja ; Sztajnbok, Flavio Roberto ; de Oliveira, Sheila Knupp Feitosa ; Campos, Lucia Maria Arruda ; Bandeira, Marcia ; Santos, Flávia Patricia Sena Teixeira ; Magalhães, Claudia Saad [UNESP]
Data: 2018
Identificador Persistente: http://hdl.handle.net/11449/172659
Origem: Oasisbr
Assunto(s): Autoinflammatory syndromes; Childhood; Familial Mediterranean fever; Fever; Guidelines
Descrição
Made available in DSpace on 2018-12-11T17:01:39Z (GMT). No. of bitstreams: 0 Previous issue date: 2016-01-01
Objective: To establish guidelines based on scientific evidence for the management of familial Mediterranean fever. Description of the evidence collection method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparisonand Outcome), to search key primary scientific information databases. After defining thepotential studies to support the recommendations, these were graduated considering theirstrength 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. Recommendations: 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)
Center for Development of Medical Teaching Medicine School Universidade de São Paulo (USP)
Pediatric Rheumatology Unit Children's Institute Medicine School Universidade de São Paulo (USP)
Hospital da Criança de Brasília José Alencar (HCB)
Irmandade da Santa Casa de Misericórdia de São Paulo
Service of Immunology Allergy and Pediatric Rheumatology Department of Pediatrics Faculdade de Medicina de Ribeirão Preto Universidade de São Paulo (USP)
Service of Rheumatology Hospital das Clínicas Universidade Federal de Pernambuco (UFPE)
Universidade Federal do Pará (UFPA)
Service of Rheumatology Nucleus Adolescents' Health Studies Universidade do Estado do Rio de Janeiro (UERJ)
Instituto de Puericultura e Pediatria Martagão Gesteira Service of Pediatric Rheumatology Universidade Federal do Rio de Janeiro (UFRJ)
Hospital Pequeno Príncipe
Service of Rheumatology Hospital das Clínicas Universidade Federal de Minas Gerais (UFMG)
Pediatric Rheumatology Unit Faculdade de Medicina de Botucatu Universidade Estadual Paulista (Unesp)
Pediatric Rheumatology Unit Faculdade de Medicina de Botucatu Universidade Estadual Paulista (Unesp)
