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O objetivo do estudo consiste em analisar os fatores que influenciaram a refratariedade ao tratamento da doença de Kawasaki com imunoglobulina endovenosa no serviço de reumatologia pediátrica do Hospital da Criança de Brasília José Alencar. A pesquisa é retrospectiva (caso-controle) e comparativa (refratários x responsivos). Foram avaliados dados epidemiológicos, clínicos e laboratoriais, sendo a única variável com relevância estatística (p= 0,006) o maior nível de plaquetas em refratários, podendo sugerir maior risco de complicações como formação de trombos. A predição de risco a refratariedade continua um desafio, necessitando de abordagem direcionada à população brasileira.

Objetivou-se descrever as características clínicas, laboratoriais e epidemiológicas da Doença de Kawasaki (DK) recorrente e identificar possíveis fatores de risco. Trata-se de estudo retrospectivo do tipo caso-controle. Foi feita análise dos prontuários de pacientes com DK acompanhados de janeiro de 2012 a dezembro de 2018 em ambulatório especializado de um hospital pediátrico terciário em Brasília - DF. Foram incluídas 154 crianças; dez (6,5%) apresentaram recidiva da DK. A incidência de recidiva encontrada foi mais alta do que consta em literatura, provavelmente devido ao acompanhamento regular em um centro especializado. Não foram identificados fatores de risco relevantes para recidiva.

Background and objectives: The present study is the first known in Latin America to enroll a substantial number of Kawasaki disease (KD) patients with an extended follow-up. This study aimed to: (1) to expose the difficulties and delays in the diagnosis of KD in a developing country, (2) to describe and correlate the clinical features of this disorder with the children’s age at the time of disease onset, (3) to correlate the frequent lack of early diagnosis with a delayed application of appropriate treatment, and (4) to describe the outcome and eventual recurrences of KD in our region. Materials and Methods: Three hundred and one participants (183 males and 118 females) included in the study were diagnosed and, subsequently, clinically followed for ten years (January 2007 to December 2016) at the Pediatric Rheumatology Walk-in Clinic of the Children’s Hospital of Brasilia. Results: Episodes ranged from four months to two years. This rate of recurrence was well-above that disclosed by previous reports. Delay in diagnosis, in all age groups, caused an undesirable delay between the disease onset, the final diagnosis, and the administration of intravenous immunoglobulin (IVIG). KD recurred in 25 (8.3%) of the children during the first three years of follow-up. In seven patients, KD recurred twice, with an interval between episodes ranging from four months to two years. Conclusions: This rate of recurrence was well-above that disclosed by previous reports. In Latin America, aside from a handful of physicians and researchers, KD is being ignored. There is a pressing need to educate primary health care physicians and bring awareness to the fact that KD is not an exotic condition that affects only the Asian populations but a disorder that already exists among us and that frequently results in severe consequences.

Made available in DSpace on 2015-12-07T15:38:44Z (GMT). No. of bitstreams: 0 Previous issue date: 2015-10-01. Added 1 bitstream(s) on 2019-10-09T18:24:49Z : No. of bitstreams: 1 S0482-50042016000100052.pdf: 646225 bytes, checksum: 1638a2e27ec46d822bf61de374bb4355 (MD5) Objetivo Estabelecer diretrizes baseadas em evidências científicas para manejo da síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA). Descrição do método de coleta de evidência A Diretriz foi elaborada a partir de cinco questões clínicas que foram estruturadas por meio do Pico (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados Foram recuperados e avaliados pelo título e resumo 806 trabalhos e selecionados 32 artigos, para sustentar as recomendações. To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. The Guideline was prepared from 5 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. 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development; 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes; 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients; 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA; 5. Despite promising results obtained in studies with IL-1ß inhibitors, such studies are limited to a few case reports. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil. Electronic address: teterreri@terra.com.br. Centro de Desenvolvimento de Educação Médica, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil. Unidade de Reumatologia Pediátrica, Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil. Hospital da Criança de Brasília José Alencar (HCB), Brasília, DF, Brasil. Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brasil. Serviço de Imunologia, Alergia e Reumatologia Pediátrica, Departamento de Pediatria, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (USP), Ribeirão Preto, SP, Brasil. Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Pernambuco (UFPE), Recife, PE, Brasil. Universidade Federal do Pará (UFPA), Belém, PA, Brasil. Serviço de Reumatologia, Núcleo de Estudos da Saúde do Adolescente, Universidade do Estado do Rio de Janeiro (Uerj), Rio de Janeiro, RJ, Brasil. Serviço de Reumatologia Pediátrica, Instituto de Puericultura e Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brasil. Hospital Pequeno Príncipe, Curitiba, PR, Brasil. Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brasil. Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brasil. Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brasil.

Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da febre familiar do Mediterrâneo (FFM). Descrição do método de coleta de evidência: A diretriz foi elaborada a partir de 5 questões clínicas que foram estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados, e avaliados pelo título e resumo, 10.341 trabalhos e selecionados 46 artigos para sustentar as recomendações. Recomendações: 1. O diagnóstico da FFM é baseado nas manifestações clínicas, caracterizadas por episódios febris recorrentes associados a dor abdominal, torácica ou artrite de grandes articulações; 2. A FFM é uma doença genética que apresenta traço autossômico recessivo ocasionada por mutação no gene MEFV; 3. Exames laboratoriais são inespecíficos e demonstram níveis séricos elevados de proteínas inflamatórias na fase aguda da doença, mas também, com frequência, níveis elevados mesmo entre os ataques. Níveis séricos de SAA podem ser especialmente úteis no monitoramento da eficácia do tratamento; 4. A colchicina é a terapia de escolha e demonstrou eficácia na prevenção dos episódios inflamatórios agudos e progressão para amiloidose em adultos; 5. Com base na informação disponível, o uso de medicamentos biológicos parece ser opção para pacientes com FFM que não respondem ou que são intolerantes à terapia com colchicina. Abstract 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, 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: 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 effectiveness in preventing acute inflammatory episodes and progression towards 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.

Kawasaki disease (KD) is one of the most common causes of childhood systemic vasculitis, with the potential to cause significant cardiovascular complications, especially if undiagnosed. An investigation of coronary artery abnormalities with the application of appropriate therapeutic measures can improve the prognosis in these infants, decreasing the risk of coronary artery aneurysms. Neonatal KD is a rare event, often characterized only by few of the clinical features observed in older children. In this case report, we describe a newborn male who presented an incomplete clinical presentation of KD, where symptoms of the disease erupted immediately after birth. The objective of this report is to draw the attention of pediatricians and neonatologists to the possible presence of KD in an asymptomatic newborn during the immediate postnatal period, even in the absence of the classic features of KD, such as fever.

Made available in DSpace on 2015-12-07T15:39:10Z (GMT). No. of bitstreams: 0 Previous issue date: 2015-10-09. Added 1 bitstream(s) on 2019-10-09T18:24:50Z : No. of bitstreams: 1 S0482-50042016000100037.pdf: 781208 bytes, checksum: b70672ad129c7cebf8eba7da1b7f87b9 (MD5) Objetivo Estabelecer diretrizes baseadas em evidências científicas para manejo da febre familiar do Mediterrâneo (FFM). Descrição do método de coleta de evidência A diretriz foi elaborada a partir de 5 questões clínicas que foram estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados Foram recuperados, e avaliados pelo título e resumo, 10.341 trabalhos e selecionados 46 artigos para sustentar as recomendações. To establish guidelines based on scientific evidence for the management of familial Mediterranean fever. The Guideline was prepared from 5 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. 10,341 articles were retrieved and evaluated by title and abstract; from these, 46 articles were selected to support the 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 effectiveness in preventing acute inflammatory episodes and progression towards 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. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil. Electronic address: teterreri@terra.com.br. Centro de Desenvolvimento de Educação Médica, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brasil. Unidade de Reumatologia Pediátrica, Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil. Hospital da Criança de Brasília José Alencar (HCB), Brasília, DF, Brasil. Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brasil. Serviço de Imunologia, Alergia e Reumatologia Pediátrica, Departamento de Pediatria, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (USP), Ribeirão Preto, SP, Brasil. Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Pernambuco (UFPE), Recife, PE, Brasil. Universidade Federal do Pará (UFPA), Belém, PA, Brasil. Serviço de Reumatologia, Núcleo de Estudos da Saúde do Adolescente, Universidade do Estado do Rio de Janeiro (Uerj), Rio de Janeiro, RJ, Brasil. Instituto de Puericultura e Pediatria Martagão Gesteira, Serviço de Reumatologia Pediátrica, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brasil. Hospital Pequeno Príncipe, Curitiba, PR, Brasil. Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brasil. Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brasil. Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (Unesp), Botucatu, SP, Brasil.

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. Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo das Sín dromes periódicas associadas à criopirina (Criopirinopatias – CAPS). Descric¸ão do método de coleta de evidência: A Diretriz foi elaborada a partir de 4 questões clínicas que foram estruturadas por meio do P.I.C.O. (Paciente, Intervenc¸ão ou Indicador, Comparac¸ão e Outcome), com busca nas principais bases primárias de informac¸ão científica. Após definir os estudos potenciais para sustento das recomendac¸ões, estes foram graduados pela forc¸a da evidência e grau de recomendac¸ão. Resultado: Foram recuperados, e avaliados pelo título e resumo, 1215 artigos, tendo sido selecionados 42 trabalhos, para sustentar as recomendac¸ões. Recomendac¸ões: 1. O diagnóstico de CAPS é baseado na anamnese e manifestac¸ões clíni cas, sendo posteriormente confirmado por estudo genético. Pode se manifestar sob três fenótipos: FCAS (forma leve), MWS (forma intermediária) e CINCA (forma grave). Avaliac¸ões neurológica, oftalmológica, otorrinolaringológica e radiológica podem ser de grande valia na distinc¸ão entre as síndromes; 2. O diagnóstico genético com análise do gene NLRP3 deve ser conduzido nos casos suspeitos de CAPS, isto é, indivíduos que apresentam, antes dos 20 anos de idade, episódios recorrentes de inflamac¸ão expressa por urticária e febre moderada; 3. As alterac¸ões laboratoriais incluem leucocitose e elevac¸ão nos níveis séricos de proteínas inflamatórias; 4. Terapias alvo dirigidas contra a interleucina 1 levam a rápida remissão dos sintomas na maioria dos pacientes. Contudo, existem limitac¸ões importantes em relac¸ão à seguranc¸a em longo prazo. Nenhuma das três medicac¸ões anti-IL1 evita progressão das lesões ósseas

BackgroundKawasaki disease (KD) is a self-limited acute systemic vasculitis of unknown aetiology that predominantly affects infants and young children eventually associated with immunological abnormalities. Coeliac disease (CD) is an inflammatory autoimmune disease characterised by a permanent gluten intolerance, which affects genetically susceptible individuals of any age group, and can cause intestinal and systemic symptoms. Association of CD with KD has been previously described in a single study that disclosed a surprisingly high prevalence of CD in children with a history of KD.ObjectiveTo confirm the existence of a higher prevalence of CD among individuals with a history of KD, which would turn the screening for CD in patients with history of KD highly advisable.SettingChildren with history of KD, diagnosed and followed at the Rheumatology Clinic of the Children’s Hospital of Brasilia (Brasilia, Brazil).ParticipantsThis study included 110 children with history of KD and a control group composed of 110 presumably healthy children.InterventionsParticipants underwent anti-transglutaminase and anti-endomysial antibodies tests and genetic typing for the presence of CD predisposing alleles (HLA-DQ2 and DQ8). Jejunal biopsy was performed when necessary, according the European Society of Paediatric Gastroenterology, Hepatology and Nutrition guidelines.ResultsDiagnosis of CD was confirmed in one (0.91%) patient with KD by positive serological tests, presence of predisposing alleles and CD typical lesions on duodenal biopsy. All serological tests were negative among the controls. The prevalence of CD predisposing alleles among patients with KD was 29.09%, similar to the prevalence found among controls, 33.64%.ConclusionThe detected CD prevalence (0.91%) does not confirm the existence of an association between KD and CD since this prevalence is similar to that found in the general population (≃1%).

Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA). Descrição do método de coleta de evidência: A Diretriz foi elaborada a partir de cinco questões clínicas que foram estruturadas por meio do Pico (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados e avaliados pelo título e resumo 806 trabalhos e selecionados 32 artigos, para sustentar as recomendações. Recomendações: 1. O diagnóstico da PFAPA é clínico e de exclusão, deve a suspeita ser considerada em crianças que apresentam episódios febris de origem indeterminada recorrentes e periódicos ou amidalites de repetição, intercalados com períodos assintomáticos, sobretudo em crianças em bom estado geral e com desenvolvimento pondero-estatural mantido; 2. Os achados laboratoriais são inespecíficos. Não existem alterações patognomônicas nos exames complementares; 3. A evidência que sustenta a indicação do tratamento cirúrgico (tonsilectomia com ou sem adenoidectomia) é baseada em dois ensaios clínicos randomizados não cegos que incluíram pequeno número de pacientes; 4. O uso de prednisona no início do quadro febril em pacientes com PFAPA mostrou ser eficaz. Melhores evidências ainda são necessárias para apoiar seu uso na PFAPA; 5. Apesar de os resultados obtidos de estudos com inibidores de IL-1ß serem promissores, esses são limitados a poucos relatos de casos. Abstract Objective: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Description of the evidence collection method: The Guideline was prepared from 5 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: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. Recommendations: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1β inhibitors, such studies are limited to a few case reports.

To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes.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.1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the 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.

Made available in DSpace on 2015-12-07T15:31:47Z (GMT). No. of bitstreams: 0 Previous issue date: 2015. Added 1 bitstream(s) on 2019-10-09T18:24:47Z : No. of bitstreams: 1 S0482-50042016000100044.pdf: 771872 bytes, checksum: 98287e6415e7d85100770f0f1070148f (MD5) Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo das síndromes periódicas associadas à criopirina (criopirinopatias – Caps). Descrição do método de coleta de evidência: A diretriz foi elaborada a partir de quatro questões clínicas que foram estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultado: Foram recuperados, e avaliados pelo título e resumo, 1.215 artigos e selecionados 42 trabalhos para sustentar as recomendações. Recomendações: 1. O diagnóstico de Caps é baseado na anamnese e nas manifestações clínicas e posteriormente confirmado por estudo genético. Pode se manifestar sob três fenótipos: FCAS (forma leve), MWS (forma intermediária) e Cinca (forma grave). Avaliações neurológica, oftalmológica, otorrinolaringológica e radiológica podem ser de grande valia na distinção entre as síndromes; 2. O diagnóstico genético com análise do gene NLRP3 deve ser conduzido nos casos suspeitos de Caps, isto é, indivíduos que apresentam, antes dos 20 anos, episódios recorrentes de inflamação expressa por urticária e febre moderada; 3. As alterações laboratoriais incluem leucocitose e elevação nos níveis séricos de proteínas inflamatórias; 4. Terapias alvo dirigidas contra a interleucina 1 levam a rápida remissão dos sintomas na maioria dos pacientes. Contudo, existem limitações importantes em relação à segurança em longo prazo. Nenhuma das três medicações anti-IL1 evita progressão das lesões ósseas. To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. 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. 1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the 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-1β inhibitors prevents progression of bone lesions. Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brasil Centro de Desenvolvimento de Educação Médica, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil Setor de Reumatologia Pediátrica, Departamento de Pediatria, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brasil Unidade de Reumatologia Pediátrica, Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brasil Hospital da Criança de Brasília José Alencar (HCB), Brasília, DF, Brasil Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brasil Serviço de Imunologia, Alergia e Reumatologia Pediátrica, Departamento de Pediatria, Faculdade de Medicina de Ribeirão Preto (FMRP), Universidade de São Paulo (USP), Ribeirão Preto, SP, Brasil Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Pernambuco (UFPE), Recife, PE, Brasil Universidade Federal do Pará (UFPA), Belém, PA, Brasil Serviço de Reumatologia, Núcleo de Estudos da Saúde do Adolescente, Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brasil Instituto de Puericultura e Pediatria Martagão Gesteira, Serviço de Reumatologia Pediátrica, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brasil Hospital Pequeno Príncipe, Curitiba, PR, Brasil Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brasil Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu (FMB), Universidade Estadual Paulista (UNESP), Botucatu, SP, Brasil Unidade de Reumatologia Pediátrica, Faculdade de Medicina de Botucatu (FMB), Universidade Estadual Paulista (UNESP), Botucatu, SP, Brasil

OBJETIVO: Chamar a atenção para as complicações, que podem surgir em qualquer fase da Doença de Kawasaki (DK), para os fatores de risco que contribuem para o aparecimento dessas complicações e para as possíveis sequelas da doença, sejam elas transitórias ou permanentes. MÉTODOS: Estudo prospectivo (coorte clínica) realizado entre abril de 2002 e abril de 2009 de 115 pacientes com DK internados no serviço de Reumatologia Pediátrica do Hospital Geral do Distrito Federal. Todos os pacientes foram sequencialmente avaliados com exames clínicos e laboratoriais, ecocardiogramas com Doppler, imitanciometria, potenciais evocados auditivos, avaliação psicológica, exame oftalmológico e, em um paciente com coreia, angiorressonância magnética cerebral. Em todos os pacientes foram aplicados questionários avaliando a possível presença de dificuldades cognitivas, emocionais, comportamentais e sociais. RESULTADOS: Vinte e cinco pacientes (21,7%) apresentaram aneurismas de coronárias. Trinta e oito pacientes (33%) apresentaram perda auditiva neurossensorial durante a doença aguda e subaguda, e 13 pacientes (11,3%) mantiveram a perda auditiva seis meses após a primeira avaliação. Outras complicações observadas foram: paralisia facial em um paciente (0,9%), ataxia na fase aguda e subaguda em 11 pacientes (9,5%), complicações oftalmológicas em 15 pacientes (13,2%), constatando-se uveíte em 13, edema de papila em um paciente e hemorragia conjuntival em outro. Um paciente apresentou coreia (0,9%) sendo que a angioressonância magnética evidenciou alterações compatíveis com isquemia cerebral. Em um paciente constatou-se a presença de aneurisma de aorta torácica (0,9%), e outro apresentou vasculite necrosante que evoluiu com gangrena periférica e perda da ponta da língua (0,9%). Alterações de comportamento durante a convalescença (20%) foi observada em 23 crianças. CONCLUSÃO: A DK pode evoluir com complicações diversas, mesmo meses após a fase aguda da doença, eventualmente resultando em sequelas permanentes. Quanto mais precoce forem o diagnóstico e a intervenção terapêutica com a administração de IgG IV, menor será a ocorrência de complicações. Presença de trombocitose, anemia e de atividade inflamatória elevada e por tempo prolongado são fatores de risco para o aparecimento de complicações. OBJECTIVE: To draw attention to complications that might arise in any Kawasaki disease (KD) stage, risk factors contributing to the onset of complications and possible transient or permanent disease sequelae. METHODS: Prospective study (clinical cohort) conducted between April 2002 and April 2009 of 115 patients with KD admitted to the Pediatric Rheumatology Clinic of the General Hospital of the Federal District, Brazil. All patients were sequentially assessed with clinical and laboratory examinations, Doppler echocardiography, imitanciometry, auditory evoked potentials, psychological evaluation, ophthalmologic examination and, in one patient with chorea, cerebral magnetic resonance angiography. In all patients, a questionnaire assessing the possible presence of cognitive, emotional, behavioral and social disorders was applied. RESULTS: Twenty-five patients (21.7%) had coronary aneurisms. Thirty eight patients (33%) had a sensorineural auditory loss during the acute and subacute phases of the disease and 13 patients (11.3%) maintained the auditory loss six months after the first assessment. Other complications observed were as follows: facial palsy in one patient (0.9%), ataxia in acute and subacute phases in 11 (9.5%); 15 patients had ophthalmologic complications (13.2%), with uveitis in 13, papilledema in one patient, and conjunctival hemorrhage in another patient. One patient experienced chorea (0.9%), with a magnetic resonance angiography showing changes consistent with cerebral ischemia. In one patient, a thoracic aorta aneurism was found (0.9%) and another patient had a necrotizing vasculitis progressing to peripheral gangrene and tongue tip loss (0.9%). Behavioral changes over convalescence were observed in 23 children. CONCLUSION: KD may progress with several complications even within months of the disease acute phase, eventually resulting in permanent sequelae. The earlier the diagnosis and therapeutic intervention with IV IgG administration are, the lower will be the occurrence of complications; the presence of thrombocytosis, anemia and elevated and extended inflammatory activity are risk factors for complication arising.

The present report describes the severe evolution of Kawasaki disease in a three-month-old infant. The ailment was initially atypical in its presentation, with the patient exhibiting only persistent fever in association with a progressive lethargy and maculopapular rash on the face, trunk and limbs erroneously diagnosed as roseola infantum. On the 10th day of the condition, mainly due to the unexplained persistence of fever, the infant was admitted to a local hospital. The typical features of KD appeared only on the 14th day of illness with the relapse of the maculopapular rash in association with non-purulent conjunctivitis; dry, reddish and fissured lips; tongue with reddish and hypertrophic papillae; erythema and edema of the palms and soles. During the following days, the ailment rapidly evolved to a catastrophic clinical picture characterized by generalized vasculitis, splenic infarction, pulmonary thrombosis, giant right and left coronary aneurysms, dilatation of common and internal iliac arteries and progressive ischemia of the distal third of the feet resulting in necrotic lesions of both halluces. Appropriate therapy was initiated, but repeated administration of intravenous immunoglobulin G (IVIG) followed by three days of administration of methylprednisolone did not abate the intense inflammatory activity. The remission of inflammation and regression of vascular lesions were only achieved during the following five weeks after the introduction of methotrexate associated with etanercept. The report of this case aims to draw attention to severe forms of KD that exhibit an unfavorable evolution and can be extremely refractory to the conventional therapy.