Guidelines for the management and treatment of periodic fever syndromes Cryopyrin-associated periodic syndromes (cryopyrinopathies - CAPS)
Carregando...
Data
2016-01-01
Autores
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
Cavalcanti, André de Souza
de Moraes, Ana Júlia Pantoja
Título da Revista
ISSN da Revista
Título de Volume
Editor
Resumo
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; and 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-1β inhibitors prevents progression of bone lesions.
Descrição
Palavras-chave
Autoinflammatory syndromes, Chronic infantile neurologic, Cutaneous and articular, Familiar cold autoinflammatory, Guidelines, Muckle-Wells syndrome, Syndrome
Como citar
Revista Brasileira de Reumatologia, v. 56, n. 1, p. 44-51, 2016.