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Title: Guidelines for the management and treatment of periodic fever syndromes cryopyrin-associated periodic syndromes (cryopyrinopathies - caps)
Diretrizes de conduta e tratamento de síndromes febris periódicas associadas à criopirina (criopirinopatias – CAPS)
Authors: Terreri, Maria Teresa R. A. [UNIFESP]
Bernardo, Wanderley Marques
Len, Claudio Arnaldo [UNIFESP]
Almeida da Silva, Clovis Artur
Ribeiro de Magalhaes, Cristina Medeiros
Sacchetti, Silvana B.
Leme Ferriani, Virginia Paes
Petry Piotto, Daniela Gerent [UNIFESP]
Cavalcanti, Andre de Souza
Pantoja de Moraes, Ana Julia
Sztajnboki, Flavio Roberto
Feitosa de Oliveira, Sheila Knupp
Arruda Campos, Lucia Maria
Bandeira, Marcia
Sena Teixeira Santos, Flavia Patricia
Magalhaes, Claudia Saad
Keywords: Familiar Cold Autoinflammatory Syndrome
Muckle-Wells Syndrome
Chronic Infantile Neurologic
Cutaneous And Articular Syndrome
Autoinflammatory Syndromes
GuidelinesMultisystem Inflammatory Disease
Cold Autoinflammatory Syndrome
Long-Term Efficacy
Sustained Response
Articular Syndrome
Cias1 Mutations
Issue Date: 2016
Publisher: Elsevier science inc
Citation: Revista Brasileira De Reumatologia. New york, v. 56, n. 1, p. 44-51, 2016.
Abstract: Objective: To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. Description of the evidence collection method: The Guideline was prepared from 4 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: 1215 articles were retrieved and evaluated by title and abstract
from these, 42 articles were selected to support the recommendations. Recommendations: 1. The diagnosis of Caps is based on clinical history and clinical manifestations, and later confirmed by genetic study. Caps may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and Cinca (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes
2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of Caps, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria
3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins
4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1 beta inhibitors prevents progression of bone lesions. (C) 2015 Elsevier Editora Ltda. All rights reserved.
ISSN: 0482-5004
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