Guidelines for the management and treatment of periodic fever syndromes: Cryopyrin-associated periodic syndromes (cryopyrinopathies - CAPS).
OBJECTIVE
To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes.


METHODS
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.


CONCLUSIONS
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.
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil. Electronic address: teterreri@terra.com.br.
Maria Teresa R A
MT
Terreri
Center for Development of Medical Teaching, Medicine School, Universidade de São Paulo (USP), São Paulo, SP, Brazil.
Wanderley Marques
WM
Bernardo
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil.
Claudio Arnaldo
CA
Len
Pediatric Rheumatology Unit, Children's Institute, Medicine School, Universidade de São Paulo (USP), São Paulo, SP, Brazil.
Clovis Artur Almeida
CA
da Silva
Hospital da Criança de Brasília José Alencar (HCB), Brasília, DF, Brazil.
Cristina Medeiros Ribeiro
CM
de Magalhães
Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brazil.
Silvana B
SB
Sacchetti
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.
Virgínia Paes Leme
VP
Ferriani
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil.
Daniela Gerent Petry
DG
Piotto
Service of Rheumatology, Hospital das Clínicas, Universidade Federal de Pernambuco (UFPE), Recife, PE, Brazil.
André de Souza
Ade S
Cavalcanti
Universidade Federal do Pará (UFPA), Belém, PA, Brazil.
Ana Júlia Pantoja
AJ
de Moraes
Service of Rheumatology, Nucleus Adolescents' Health Studies, Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil.
Flavio Roberto
FR
Sztajnbok
Instituto de Puericultura e Pediatria Martagão Gesteira, Service of Pediatric Rheumatology, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil.
Sheila Knupp Feitosa
SK
de Oliveira
Pediatric Rheumatology Unit, Children's Institute, Medicine School, Universidade de São Paulo (USP), São Paulo, SP, Brazil.
Lucia Maria Arruda
LM
Campos
Hospital Pequeno Príncipe, Curitiba, PR, Brazil.
Marcia
M
Bandeira
Service of Rheumatology, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.
Flávia Patricia Sena Teixeira
FP
Santos
Pediatric Rheumatology Unit, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brazil.
Claudia Saad
CS
Magalhães
D017668:Age of Onset; D056587:Cryopyrin-Associated Periodic Syndromes / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D019317:Evidence-Based Medicine; D005334:Fever; D006801:Humans; D007249:Inflammation / Q000235:genetics / Q000276:immunology; D053583:Interleukin-1beta; D009154:Mutation; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein / Q000235:genetics*; D017410:Practice Guidelines as Topic; D011379:Prognosis; D012720:Severity of Illness Index; D014581:Urticaria
D053583:Interleukin-1beta; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human
Autoinflammatory syndromes; Chronic infantile neurologic Cutaneous and articular syndrome; Diretrizes; Familiar cold autoinflammatory syndrome; Guidelines; Muckle-Wells syndrome; Síndrome autoinflamatória familiar associada ao frio; Síndrome de Muckle-Wells; Síndrome neurológica cutânea e articular crônica infantil; Síndromes autoinflamatórias