Guidelines for the management and treatment of periodic fever syndromes cryopyrin-associated periodic syndromes (cryopyrinopathies - caps)

Guidelines for the management and treatment of periodic fever syndromes cryopyrin-associated periodic syndromes (cryopyrinopathies - caps)

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)
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
Pantoja de Moraes, Ana Julia Google Scholar
Sztajnboki, 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 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.
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
Language Portuguese
Date 2016
Published in Revista Brasileira De Reumatologia. New york, v. 56, n. 1, p. 44-51, 2016.
ISSN 0482-5004 (Sherpa/Romeo, impact factor)
Publisher Elsevier science inc
Extent 44-51
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000374895300008
SciELO ID S0482-50042016000100044 (statistics in SciELO)

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