Guidelines for the management and treatment of periodic fever syndromes familial Mediterranean fever.

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; de Souza Cavalcanti, André; 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

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; de Souza Cavalcanti, André; 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.

Afiliación

Terreri MT; Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil. Electronic address: teterreri@terra.com.br.
Bernardo WM; Center for Development of Medical Teaching, Medicine School, Universidade de São Paulo (USP), São Paulo, SP, Brazil.
Len CA; Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil.
da Silva CA; Pediatric Rheumatology Unit, Children's Institute, Medicine School, Universidade de São Paulo (USP), São Paulo, SP, Brazil.
de Magalhães CM; Hospital da Criança de Brasília José Alencar (HCB), Brasília, DF, Brazil.
Sacchetti SB; Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brazil.
Ferriani VP; 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.
Piotto DG; Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil.
de Souza Cavalcanti A; Service of Rheumatology, Hospital das Clínicas, Universidade Federal de Pernambuco (UFPE), Recife, PE, Brazil.
de Moraes AJ; Universidade Federal do Pará (UFPA), Belém, PA, Brazil.
Sztajnbok FR; Service of Rheumatology, Nucleus Adolescents' Health Studies, Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil.
de Oliveira SK; Instituto de Puericultura e Pediatria Martagão Gesteira, Service of Pediatric Rheumatology, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil.
Campos LM; Pediatric Rheumatology Unit, Children's Institute, Medicine School, Universidade de São Paulo (USP), São Paulo, SP, Brazil.
Bandeira M; Hospital Pequeno Príncipe, Curitiba, PR, Brazil.
Santos FP; Service of Rheumatology, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.
Magalhães CS; Pediatric Rheumatology Unit, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brazil.

Rev Bras Reumatol Engl Ed ; 56(1): 37-43, 2016.

Article en En, Pt | MEDLINE | ID: mdl-27267332

RESUMEN

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 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 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.

Asunto(s)

Amiloidosis Familiar/prevención & control; Colchicina/uso terapéutico; Fiebre Mediterránea Familiar/diagnóstico; Fiebre Mediterránea Familiar/terapia; Guías de Práctica Clínica como Asunto; Pirina/genética; Amiloidosis Familiar/genética; Medicina Basada en la Evidencia; Fiebre Mediterránea Familiar/genética; Humanos; Fenotipo; Síndrome

Palabras clave

Autoinflammatory syndromes; Childhood; Diretrizes; Familial Mediterranean fever; Febre; Febre familiar do Mediterrâneo; Fever; Guidelines; Infância; Síndromes autoinflamatórias

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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fiebre Mediterránea Familiar / Colchicina / Guías de Práctica Clínica como Asunto / Amiloidosis Familiar / Pirina Tipo de estudio: Guideline Límite: Humans Idioma: En / Pt Revista: Rev Bras Reumatol Engl Ed Año: 2016 Tipo del documento: Article Pais de publicación: Brasil

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