Guidelines for the management and treatment of periodic fever syndromes familial mediterranean fever

Guidelines for the management and treatment of periodic fever syndromes familial mediterranean fever

Title: Guidelines for the management and treatment of periodic fever syndromes familial mediterranean fever;
Diretrizes de conduta e tratamento de síndromes febris periódicas associadas a febre familiar do Mediterrâneo
Author Terreri, Maria Teresa R. A. Autor UNIFESP Google Scholar
Bernardo, Wanderley Marques Google Scholar
Len, Claudio Arnaldo Autor UNIFESP Google Scholar
Almeida da Silva, Clovis Artur Google Scholar
Ribeiro de Magalhaes, Cristina Medeiros Google Scholar
Sacchetti, Silvana B. Google Scholar
Leme Ferriani, Virginia Paes Google Scholar
Petry Piotto, Daniela Gerent Autor UNIFESP Google Scholar
Cavalcanti, Andre de Souza Google Scholar
de Moraes, Ana Julia Pantoja Google Scholar
Sztajnbok, Flavio Roberto Google Scholar
Feitosa de Oliveira, Sheila Knupp Google Scholar
Arruda Campos, Lucia Maria Google Scholar
Bandeira, Marcia Google Scholar
Sena Teixeira Santos, Flavia Patricia Google Scholar
Magalhaes, Claudia Saad Google Scholar
Abstract 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, 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: 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 effectiveness in preventing acute inflammatory episodes and progression towards 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. (C) 2015 Elsevier Editora Ltda. All rights reserved.
Keywords Familial Mediterranean Fever
Guidelines
Childhood
Fever
Autoinflammatory SyndromesAutoinflammatory Syndromes
Colchicine Therapy
Amyloidosis
Activation
Genotype
Patient
Attacks
Transplantation
Interleukin-1
Canakinumab
Language Portuguese
Date 2016
Published in Revista Brasileira De Reumatologia. New york, v. 56, n. 1, p. 37-43, 2016.
ISSN 0482-5004 (Sherpa/Romeo, impact factor)
Publisher Elsevier science inc
Extent 37-43
Origin http://dx.doi.org/10.1016/j.rbr.2015.08.006
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000374895300007
SciELO ID S0482-50042016000100037 (statistics in SciELO)
URI http://repositorio.unifesp.br/handle/11600/49530

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