Artículos de revistas
Guidelines for the management and treatment of periodic fever syndromes familial Mediterranean fever
Fecha
2016-01-01Registro en:
Revista Brasileira de Reumatologia, v. 56, n. 1, p. 37-43, 2016.
1809-4570
0482-5004
10.1016/j.rbre.2015.08.019
2-s2.0-84960361550
2-s2.0-84960361550.pdf
7098310008371632
0000-0002-7631-7093
Autor
Universidade Federal de São Paulo (UNIFESP)
Universidade de São Paulo (USP)
Hospital da Criança de Brasília José Alencar (HCB)
Irmandade da Santa Casa de Misericórdia de São Paulo
Universidade Federal de Pernambuco (UFPE)
Universidade Federal do Pará (UFPA)
Universidade do Estado do Rio de Janeiro (UERJ)
Universidade Federal do Rio de Janeiro (UFRJ)
Hospital Pequeno Príncipe
Universidade Federal de Minas Gerais (UFMG)
Universidade Estadual Paulista (Unesp)
Institución
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, 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.