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Abstract This research evaluated the combined effects of instructions (through health education manual) and self-monitoring (using record forms) in occurrence of adherence to treatment by adolescents diagnosed with Juvenile Systemic Lupus Erythematosus. Four adolescents participated in the research, which had four stages: 1. Initial assessment; 2. Intervention, including Condition 1, with exposure to the manual followed by the self-monitoring record forms, and Condition 2, with a reversal order of these instruments; 3. Feedback; and 4. Follow-up. The data obtained with 24h recall and self-monitoring record forms were quantified, allowing the calculation of the Treatment Adherence Index. Three participants showed an increase in this index after the use of self-monitoring records, regardless the exposition order of the instruments. The results of this research suggest that self-monitoring can assist in the installation of adherence behaviors, while manuals with instructions can help to maintain those behaviors, which would justify the combined use of these instruments.

Resumo Esta pesquisa avaliou os efeitos do uso combinado de instruções (via manual de educação em saúde) e de automonitorização (via formulários de registro) sobre a ocorrência de relatos de adesão ao tratamento por adolescentes com diagnóstico de Lúpus Juvenil. Quatro adolescentes participaram da pesquisa realizada em quatro etapas: 1. Avaliação inicial; 2. Intervenção, incluindo Condição 1, com exposição ao Manual seguido dos Formulários de Registro de Automonitorização, e Condição 2, com inversão da aplicação dos instrumentos; 3. Feedback; e 4. Follow-up. Os dados obtidos por meio de Recordatórios 24h e de registros em Formulários de Automonitorização foram quantificados, permitindo o cálculo do Índice de Adesão ao Tratamento. Três participantes melhoraram a adesão após a automonitorização, independentemente da ordem de apresentação dos instrumentos. Os resultados sugerem que a automonitorização pode auxiliar na instalação de comportamentos de adesão, enquanto o manual com instruções pode auxiliar na manutenção dos mesmos, o que justificaria o uso combinado destes instrumentos.

Resumen Esta investigación tuvo por objetivo evaluar los efectos del uso combinado de instrucciones (vía manual de educación en salud) y de automonitorización (vía formularios de registro) sobre la ocurrencia de relatos de adhesión al tratamiento por adolescentes con diagnóstico de Lupus Juvenil. Cuatro adolescentes participaron de la investigación que se realizó en cuatro etapas: 1. evaluación inicial; 2. Intervención, incluyendo - Condición 1, con exposición al Manual seguido de los Formularios de Registro de Automonitorización, y Condición 2, con inversión de la aplicación de los instrumentos; 3. Feedback; 4. Follow-up. Los datos obtenidos a partir del recordatorio de 24h y de registros en los Formularios de Automonitorización fueron cuantificados, permitiendo el cálculo del Índice de Adhesión al Tratamiento. Tres participantes mejoraron la adhesión después de la automonitorización, independientemente del orden de presentación de los instrumentos. Los resultados sugieren que la automonitorización puede auxiliar en la instalación de comportamientos de adhesión, mientras que el manual con instrucciones puede auxiliar en el mantenimiento de los mismos, lo que justificaría el uso combinado de estos instrumentos.

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RESUMO Descreve-se o estudo realizado com duas adolescentes (P1 e P2) com diagnóstico de lúpus acompanhadas em ambulatório de reumatologia. Investigaram-se os efeitos do uso de registros de automonitorização (RA) e de recordatório 24 horas (R24h) sobre o índice de adesão ao tratamento (IAT) por meio de entrevistas semiestruturadas. P1 foi submetida à sequência R24h-RA e P2, à ordem inversa. Em linha de base, ambas obtiveram valores de IAT abaixo ou igual a 50%. Após intervenção, alcançaram valores acima de 71%, indicando melhora no índice de adesão independentemente da ordem em que os instrumentos foram apresentados. Discute-se o controle do comportamento por regras e a importância da instalação de repertórios de relatos fidedignos sobre comportamentos de adesão ao tratamento do lúpus

ABSTRACT This article describes a study conducted with two teenagers (P1 and P2) who were diagnosed with lupus and attended a rheumatology clinic. The aim was to investigate the effects of the use of a self-monitoring registry (RA) and a 24h recall (R24h) on the rate of Treatment Adherence Index (IAT) through semi-structured interviews. Participant P1 was submitted to a 24h-RA sequence whereas participant P2 to the reverse order. At baseline, both of them had IAT rates below or equal to 50%; after the intervention, they achieved rates above 71%, showing improvements in adherence. The control of behavior by rules and the importance of installing a set of reliable accounts of behavior of adherence to the treatment of lupus were discussed.

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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 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 its effectiveness in preventing acute inflammatory episodes and progression toward 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.

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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-1ß inhibitors prevents progression of bone lesions.

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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-1ß inhibitors, such studies are limited to a few case reports.

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OBJECTIVE To estimate the prevalence of sexual initiation and contraceptive use at the last sexual intercourse of Brazilian adolescents, according to sociodemographic features. METHODS The data were obtained from the Study of Cardiovascular Risks in Adolescents (ERICA), a national school-based cross-sectional study. We included 74,589 adolescents from 32 geographic strata (27 capitals and five sets of municipalities with more than 100,000 inhabitants of each of the five macro-regions of the Country). Information on sexual initiation and contraceptive use at the last sexual intercourse (male condom and oral contraceptive pill) has been used. We have estimated prevalence and confidence intervals (95%CI) considering sample weights according to sex, age, type of school, residence status, macro-region and capitals. RESULTS We observed that 28.1% (95%CI 27.0-29.2) of the adolescents had already initiated sexual life, with higher prevalence among those aged 17 years (56.4%, 95%CI 53.9-58.9), males (33.5%, 95%CI 31.8-35.2), studying at public schools (29.9%, 95%CI 28.5-31.4), and from the Northern region (33.9%, 95%CI 32.3-35.4), mainly from Macapa, Manaus, and Rio Branco. Among those who had started their sexual life, 82.3% (95%CI 81.1-83.4) reported the use of contraceptive methods at the last intercourse, and the prevalence of use was higher among adolescents aged 17 years (85.3%, 95%CI 82.7-87.6), females (85.2%, 95%CI 83.8-86.5) and those living in the Southern region (85.9%, 95%CI 82.9-88.5). Male condom was used by 68.8% (95%CI 66.9-70.7), with no difference by type of school or macro-regions; the contraceptive pill was used by 13.4% (CI95% 12.2-14.6), and more frequently used among women (24.7%, 95%CI 22.5-27,0) and 17-year-old adolescents (20.8%, 95%CI 18.2-23.6) from urban settings(13.7%, 95%CI 12.5-14.9) and from the Southern region (22.6%, 95%CI 19.0-26.8), and less often in the Northern region. CONCLUSIONS ERICA's data analysis on sexuality and contraception shows heterogeneities in the prevalence of sexual initiation and use of contraceptive methods among Brazilian adolescents, depending on their age, where they live, and the type of school they study at. Younger adolescents and those living in the Northern region seem to be more vulnerable to the consequences of unprotected sexual intercourses.

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OBJECTIVE To determine the prevalence of metabolic syndrome and its components in Brazilian adolescents. METHODS We evaluated 37,504 adolescents who were participants in the Study of Cardiovascular Risks in Adolescents (ERICA), a cross-sectional, school-based, national study. The adolescents, aged from 12 to 17 years, lived in cities with populations greater than 100,000 inhabitants. The sample was stratified and clustered into schools and classes. The criteria set out by the International Diabetes Federation were used to define metabolic syndrome. Prevalences of metabolic syndrome were estimated according to sex, age group, school type and nutritional status. RESULTS Of the 37,504 adolescents who were evaluated: 50.2% were female; 54.3% were aged from 15 to 17 years, and 73.3% were from public schools. The prevalence of metabolic syndrome was 2.6% (95%CI 2.3-2.9), slightly higher in males and in those aged from 15 to 17 years in most macro-regions. The prevalence was the highest in residents from the South macro-region, in the younger female adolescents and in the older male adolescents. The prevalence was higher in public schools (2.8% [95%CI 2.4-3.2]), when compared with private schools (1.9% [95%CI 1.4-2.4]) and higher in obese adolescents when compared with nonobese ones. The most common combinations of components, referring to 3/4 of combinations, were: enlarged waist circumference (WC), low HDL-cholesterol (HDL-c) and high blood pressure; followed by enlarged WC, low HDL-c and high triglycerides; and enlarged WC, low HDL-c, high triglycerides and blood pressure. Low HDL was the second most frequent component, but the highest prevalence of metabolic syndrome (26.8%) was observed in the presence of high triglycerides. CONCLUSIONS ERICA is the first Brazilian nation-wide study to present the prevalence of metabolic syndrome and describe the role of its components. Despite the prevalence of Metabolic Syndrome being low, the high prevalences of some components and participation of others in the syndrome composition shows the importance of early diagnosis of this changes, even if not grouped within the metabolic syndrome.

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OBJECTIVE To estimate the prevalence of arterial hypertension and obesity and the population attributable fraction of hypertension that is due to obesity in Brazilian adolescents. METHODS Data from participants in the Brazilian Study of Cardiovascular Risks in Adolescents (ERICA), which was the first national school-based, cross-section study performed in Brazil were evaluated. The sample was divided into 32 geographical strata and clusters from 32 schools and classes, with regional and national representation. Obesity was classified using the body mass index according to age and sex. Arterial hypertension was defined when the average systolic or diastolic blood pressure was greater than or equal to the 95th percentile of the reference curve. Prevalences and 95% confidence intervals (95%CI) of arterial hypertension and obesity, both on a national basis and in the macro-regions of Brazil, were estimated by sex and age group, as were the fractions of hypertension attributable to obesity in the population. RESULTS We evaluated 73,399 students, 55.4% female, with an average age of 14.7 years (SD = 1.6). The prevalence of hypertension was 9.6% (95%CI 9.0-10.3); with the lowest being in the North, 8.4% (95%CI 7.7-9.2) and Northeast regions, 8.4% (95%CI 7.6-9.2), and the highest being in the South, 12.5% (95%CI 11.0-14.2). The prevalence of obesity was 8.4% (95%CI 7.9-8.9), which was lower in the North region and higher in the South region. The prevalences of arterial hypertension and obesity were higher in males. Obese adolescents presented a higher prevalence of hypertension, 28.4% (95%CI 25.5-31.2), than overweight adolescents, 15.4% (95%CI 17.0-13.8), or eutrophic adolescents, 6.3% (95%CI 5.6-7.0). The fraction of hypertension attributable to obesity was 17.8%. CONCLUSIONS ERICA was the first nationally representative Brazilian study providing prevalence estimates of hypertension in adolescents. Regional and sex differences were observed. The study indicates that the control of obesity would lower the prevalence of hypertension among Brazilian adolescents by 1/5.

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ABSTRACT OBJECTIVE To estimate the prevalence of sexual initiation and contraceptive use at the last sexual intercourse of Brazilian adolescents, according to sociodemographic features. METHODS The data were obtained from the Study of Cardiovascular Risks in Adolescents (ERICA), a national school-based cross-sectional study. We included 74,589 adolescents from 32 geographic strata (27 capitals and five sets of municipalities with more than 100,000 inhabitants of each of the five macro-regions of the Country). Information on sexual initiation and contraceptive use at the last sexual intercourse (male condom and oral contraceptive pill) has been used. We have estimated prevalence and confidence intervals (95%CI) considering sample weights according to sex, age, type of school, residence status, macro-region and capitals. RESULTS We observed that 28.1% (95%CI 27.0-29.2) of the adolescents had already initiated sexual life, with higher prevalence among those aged 17 years (56.4%, 95%CI 53.9-58.9), males (33.5%, 95%CI 31.8-35.2), studying at public schools (29.9%, 95%CI 28.5-31.4), and from the Northern region (33.9%, 95%CI 32.3-35.4), mainly from Macapa, Manaus, and Rio Branco. Among those who had started their sexual life, 82.3% (95%CI 81.1-83.4) reported the use of contraceptive methods at the last intercourse, and the prevalence of use was higher among adolescents aged 17 years (85.3%, 95%CI 82.7-87.6), females (85.2%, 95%CI 83.8-86.5) and those living in the Southern region (85.9%, 95%CI 82.9-88.5). Male condom was used by 68.8% (95%CI 66.9-70.7), with no difference by type of school or macro-regions; the contraceptive pill was used by 13.4% (CI95% 12.2-14.6), and more frequently used among women (24.7%, 95%CI 22.5-27,0) and 17-year-old adolescents (20.8%, 95%CI 18.2-23.6) from urban settings(13.7%, 95%CI 12.5-14.9) and from the Southern region (22.6%, 95%CI 19.0-26.8), and less often in the Northern region. CONCLUSIONS ERICA’s data analysis on sexuality and contraception shows heterogeneities in the prevalence of sexual initiation and use of contraceptive methods among Brazilian adolescents, depending on their age, where they live, and the type of school they study at. Younger adolescents and those living in the Northern region seem to be more vulnerable to the consequences of unprotected sexual intercourses.

RESUMO OBJETIVO Estimar prevalências de iniciação sexual e uso de métodos contraceptivos na última relação sexual de adolescentes brasileiros, segundo características sociodemográficas. MÉTODOS Os dados foram obtidos do Estudo de Riscos Cardiovasculares em Adolescentes (ERICA), estudo transversal nacional de base escolar. Foram incluídos 74.589 adolescentes provenientes de 32 estratos geográficos (27 capitais e cinco conjuntos de municípios com mais de 100 mil habitantes de cada uma das cinco macrorregiões do País). Utilizaram-se informações sobre iniciação sexual e uso de métodos contraceptivos na última relação sexual (preservativo masculino e pílula anticonceptiva oral). Estimaram-se prevalências e intervalos de confiança (IC95%) das variáveis de interesse considerando pesos amostrais e segundo sexo, idade, tipo de escola, situação de residência, macrorregião e capitais. RESULTADOS Observou-se que 28,1% (IC95% 27,0-29,2) dos adolescentes tinham iniciado a vida sexual, com maior prevalência naqueles com 17 anos (56,4%, IC95% 53,9-58,9), no sexo masculino (33,5%, IC95% 31,8-35,2), em escolas públicas (29,9%, IC95% 28,5-31,4) e na região Norte (33,9%, IC95% 32,3-35,4), destacando-se em Macapá, Manaus e Rio Branco. Entre adolescentes que tinham iniciado a vida sexual, 82,3% (IC95% 81,1-83,4) referiram uso de métodos contraceptivos na última relação sexual, sendo a prevalência de uso maior entre adolescentes com 17 anos de idade (85,3%, IC95% 82,7-87,6), mulheres (85,2%, IC95%:83,8-86,5) e residentes na região Sul (85,9%, IC95% 82,9-88,5). O preservativo masculino foi usado por 68,8% (IC95% 66,9-70,7), sem diferença por tipo de escola ou macrorregiões. Pílula anticoncepcional foi utilizada por 13,4% (IC95% 12,2-14,6), sendo mais frequente entre mulheres (24,7%, IC95% 22,5-27,0), adolescentes de 17 anos (20,8%, IC95% 18,2-23,6), da área urbana (13,7%, IC95% 12,5-14,9) e da região Sul (22,6%, IC95% 19,0-26,8), e menos frequente na região Norte. CONCLUSÕES A análise dos dados sobre sexualidade e contracepção do ERICA mostra que há heterogeneidades nas prevalências de iniciação sexual e uso de métodos contraceptivos entre os adolescentes brasileiros, a depender de sua idade, de onde vivem e do tipo de escola que frequentam. Adolescentes mais novos e residentes na região Norte parecem ser os mais vulneráveis às consequências das práticas sexuais não protegidas.

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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.

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Resumo 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-IL1β evita progressão das lesões ósseas.

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; 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.

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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.

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Lúpus eritematoso sistêmico juvenil (LESJ) é uma doença crônica diagnosticada em crianças e adolescentes cujo tratamento requer o seguimento de regras complexas, demandando educação em saúde. Este estudo descreve o processo de elaboração e avaliação de um manual de orientações para pacientes com LESJ. Participaram onze profissionais da área de saúde e três crianças com LESJ e suas mães, em quatro etapas de avaliação do manual, elaborado a partir de bibliografia especializada. Na primeira etapa, realizada com quatro reumatologistas, 70% do texto foi alterado. Na segunda, com quatro psicólogos, 65,5%. Na terceira, com três outros psicólogos, texto e ilustrações foram avaliado sem conjunto, verificando-se a compreensibilidade e clareza do material. Os resultados obtidos demonstraram que cinco dos 12 temas (41,66%) obtiveram 100% de concordância entre os participantes em todos os critérios avaliados.Os dados obtidos nesta etapa demonstraram boa aceitação do material, uma vez que, mesmo nos temas que não obtiveram 100% de concordância em todos os itens, o percentual de desacordo foi baixo. Na quarta etapa, realizada com três crianças e suas cuidadoras, os participantes obtiveram 100% de acerto ao responderem questionário após a leitura do manual, mesmo demonstrando desconhecimento da doença. O resultado foi uma versão final do manual tendo como público-alvo a população infanto-juvenil...

Juvenile Systemic Lupus Erythematosus (JSLE) is a chronic disease diagnosed in children and adolescents. Its treatment requires the following of complex rules and demands health education. This study describes the development processand evaluation of a Guidelines Manual for patients with JSLE. Participants were eleven health professionals, three children with JSLE and their mothers, in four manual evaluation steps, drawn from specialized literature. In the first stage, with four rheumatologists, 70% of the text was changed. On the second, with four psychologists, 65.5%. In the third, with three other psychologists, both text and illustrations were evaluated to check the text’s understandability and clarity. The results showed that five of the 12 subjects (41.66%) achieved 100% agreement among the participants in all evaluated criteria. The data obtained in this step showed good acceptance of the material, since, even in subjects that did not get 100% agreement on all items, the disagreement percentage was low. In the fourth stage, performed with three children and their caregivers, participants had 100% accuracy when answering a questionnaire after reading the manual, even when ignoring the disease. The result was a final version of the manual whose target audience was children and adolescents...

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Lúpus eritematoso sistêmico juvenil (LESJ) é uma doença crônica diagnosticada em crianças e adolescentes cujo tratamento requer o seguimento de regras complexas, demandando educação em saúde. Este estudo descreve o processo de elaboração e avaliação de um manual de orientações para pacientes com LESJ. Participaram onze profissionais da área de saúde e três crianças com LESJ e suas mães, em quatro etapas de avaliação do manual, elaborado a partir de bibliografia especializada. Na primeira etapa, realizada com quatro reumatologistas, 70% do texto foi alterado. Na segunda, com quatro psicólogos, 65,5%. Na terceira, com três outros psicólogos, texto e ilustrações foram avaliado sem conjunto, verificando-se a compreensibilidade e clareza do material. Os resultados obtidos demonstraram que cinco dos 12 temas (41,66%) obtiveram 100% de concordância entre os participantes em todos os critérios avaliados.Os dados obtidos nesta etapa demonstraram boa aceitação do material, uma vez que, mesmo nos temas que não obtiveram 100% de concordância em todos os itens, o percentual de desacordo foi baixo. Na quarta etapa, realizada com três crianças e suas cuidadoras, os participantes obtiveram 100% de acerto ao responderem questionário após a leitura do manual, mesmo demonstrando desconhecimento da doença. O resultado foi uma versão final do manual tendo como público-alvo a população infanto-juvenil. (AU)

Juvenile Systemic Lupus Erythematosus (JSLE) is a chronic disease diagnosed in children and adolescents. Its treatment requires the following of complex rules and demands health education. This study describes the development processand evaluation of a Guidelines Manual for patients with JSLE. Participants were eleven health professionals, three children with JSLE and their mothers, in four manual evaluation steps, drawn from specialized literature. In the first stage, with four rheumatologists, 70% of the text was changed. On the second, with four psychologists, 65.5%. In the third, with three other psychologists, both text and illustrations were evaluated to check the text’s understandability and clarity. The results showed that five of the 12 subjects (41.66%) achieved 100% agreement among the participants in all evaluated criteria. The data obtained in this step showed good acceptance of the material, since, even in subjects that did not get 100% agreement on all items, the disagreement percentage was low. In the fourth stage, performed with three children and their caregivers, participants had 100% accuracy when answering a questionnaire after reading the manual, even when ignoring the disease. The result was a final version of the manual whose target audience was children and adolescents. (AU)

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BACKGROUND: The Study of Cardiovascular Risk in Adolescents (Portuguese acronym, "ERICA") is a multicenter, school-based country-wide cross-sectional study funded by the Brazilian Ministry of Health, which aims at estimating the prevalence of cardiovascular risk factors, including those included in the definition of the metabolic syndrome, in a random sample of adolescents aged 12 to 17 years in Brazilian cities with more than 100,000 inhabitants. Approximately 85,000 students were assessed in public and private schools. Brazil is a continental country with a heterogeneous population of 190 million living in its five main geographic regions (North, Northeast, Midwest, South and Southeast). ERICA is a pioneering study that will assess the prevalence rates of cardiovascular risk factors in Brazilian adolescents using a sample with national and regional representativeness. This paper describes the rationale, design and procedures of ERICA. METHODS/DESIGN: Participants answered a self-administered questionnaire using an electronic device, in order to obtain information on demographic and lifestyle characteristics, including physical activity, smoking, alcohol intake, sleeping hours, common mental disorders and reproductive and oral health. Dietary intake was assessed using a 24-hour dietary recall. Anthropometric measures (weight, height and waist circumference) and blood pressure were also be measured. Blood was collected from a subsample of approximately 44,000 adolescents for measurements of fasting glucose, total cholesterol, HDL-cholesterol, LDL-cholesterol, triglycerides, glycated hemoglobin and fasting insulin. DISCUSSION: The study findings will be instrumental to the development of public policies aiming at the prevention of obesity, atherosclerotic diseases and diabetes in an adolescent population.

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OBJETIVO: Avaliar a saúde reprodutiva de homens com miopatia inflamatória idiopática (MII) e compará-la com controles saudáveis. MÉTODOS: Vinte e cinco pacientes com MII (dermatomiosite ou polimiosite) foram avaliados com relação aos dados demográficos, exame urológico (incluindo parâmetros pubertários e função sexual/erétil), ultrassonografia testicular, perfil hormonal, análise seminal, características clínicas e tratamento. O grupo controle incluiu 25 homens saudáveis. RESULTADOS: A mediana da idade atual foi similar nos pacientes com MII e controles (24 versus 27 anos, P = 0,566). As frequências de atividade sexual, número de parceiras com gestações espontâneas após início da doença e uso de preservativo masculino foram significativamente menores nos pacientes com MII versus controles (60 por cento versus 96 por cento, P = 0,004; 16 por cento versus 60 por cento, P = 0,0031; 40 por cento versus 76 por cento, P = 0,021; respectivamente). Além disso, as frequências de atrofia testicular (28 por cento versus 4 por cento, P = 0,049), níveis elevados de FSH e/ou LH (25 por cento versus 0 por cento, P = 0,05) e alterações dos espermatozoides (40 por cento versus 0 por cento, P = 0,0006) foram estatisticamente maiores nos pacientes com MII quando comparados aos controles. As medianas das idades de início da doença e atual foram estatisticamente maiores nos pacientes com MII que apresentaram disfunção sexual/erétil versus sem disfunção (41 versus 12,5 anos, P = 0,014; 46 versus 21 anos, P = 0,027; respectivamente). Entretanto, comparando-se, pacientes com disfunção sexual/erétil e sem disfunção, nenhuma diferença foi evidenciada em relação à idade da espermarca, parâmetros de função gonadal, atividade da doença, enzimas musculares e tratamento...

OBJECTIVE: To evaluate reproductive health of males with idiopathic inflammatory myopathies (IIM), and comparing them with a control group. METHODS: Demographic data, urologic evaluation (including pubertal parameters and sexual/erectile function), testicular ultrasound, hormone profile, semen analysis, clinical features, and treatment of 25 IIM patients were evaluated. The control group was composed of 25 healthy males. RESULTS: Median age of IIM patients was similar to that of the control group (24 versus 27 years, P = 0.566). The frequency of sexual activity, number of partners with spontaneous pregnancies after the onset of the disease, and use of condom were significantly lower in IIM patients than in the control group (60 percent versus 96 percent, P = 0.004; 16 percent versus 60 percent, P = 0.0031; 40 percent versus 76 percent, P = 0.021, respectively). Moreover, the frequency of testicular atrophy (28 percent versus 4 percent, P = 0.049), elevated levels of FSH and/or LH (25 percent versus 0 percent, P = 0.05), and sperm abnormalities (40 percent versus 0 percent, P = 0.0006) were statistically higher in IIM patients than in the control group. Median age of onset of IIM and current age were significantly higher in IIM patients with sexual/erectile dysfunction than in patients without this dysfunction (41 versus 12.5 years, P = 0.014; 46 versus 21 years, P = 0.027, respectively). On the other hand, differences in the age of spermarche, parameters of gonadal function, disease activity, muscle enzymes, and treatment were not observed between IIM patients with or without sexual/erectile dysfunction...

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OBJETIVO: Avaliar a saúde reprodutiva em homens com lúpus eritematoso sistêmico (LES) e compará-la com controles saudáveis. MÉTODOS: Vinte e cinco pacientes com LES do sexo masculino foram avaliados com dados demográficos, exame urológico (incluindo parâmetros pubertários e função sexual/erétil), ultrassonografia testicular com Doppler, perfil hormonal, análise seminal, características clínicas e tratamento. O grupo-controle incluiu 25 homens saudáveis. RESULTADOS: A mediana da idade atual foi similar nos pacientes com LES comparada aos controles (26 versus 27 anos, P = 0,756). As frequências de disfunções sexual/erétil foram significativamente maiores nos pacientes com LES em relação aos controles (20 por cento versus 0 por cento, P = 0,0001) e o número de gestações espontâneas foi menor (20 por cento versus 60 por cento, P = 0,0086). Uma tendência de uso infrequente de contraceptivos foi observada em pacientes com LES comparada aos controles (48 por cento versus 76 por cento, P = 0,079). Além disso, as frequências de parâmetros de disfunção gonadal: atrofia testicular avaliada pela ultrassonografia (36 por cento versus 8 por cento, P = 0,037), níveis elevados de FSH e/ou LH (36 por cento versus 0 por cento, P = 0,002) e alterações dos espermatozoides (48 por cento versus 0 por cento, P = 0,0001) foram estatisticamente maiores nos pacientes com LES versus controles. Os pacientes com LES e disfunção sexual/erétil não realizaram atividade sexual no último mês versus 95 por cento dos pacientes sem disfunção (P = 0,0001). Entretanto, nenhuma diferença foi evidenciada nos pacientes com LES com e sem disfunção sexual/erétil em relação a dados demográficos, atividade da doença, dano cumulativo e tratamento. CONCLUSÃO: Este é o primeiro estudo que identificou disfunção sexual/erétil e gonadal em homens lúpicos. Uma abordagem multidisciplinar é essencial para oferecer medidas preventivas para esses pacientes.

OBJECTIVE: To assess reproductive health in male systemic lupus erythematosus (SLE) patients and compare them with controls. METHODS: Twenty-five male SLE patients were evaluated for demographic data, urologic evaluation (including pubertal parameters, sexual/erectile function), testicular Doppler ultrasound, hormone profile, semen analysis, clinical features and treatment. The control group included 25 healthy men. RESULTS: The current median age was similar in SLE patients compared with controls (26 versus 27 years, P = 0.756). The frequencies of sexual/erectile disfunction were significantly higher (20 percent versus 0 percent, P = 0.0001) and the number of spontaneous pregnancies were lower in SLE patients than in controls (20 percent versus 60 percent, P = 0.0086). A trend to low contraceptive use was observed in SLE patients compared with controls (48 percent versus 76 percent, P = 0.079). Moreover, the frequencies of gonadal dysfunction parameters, such as testicular atrophies measured by ultrasound (36 percent versus 8 percent, P = 0.037), elevated FSH and/or LH levels (36 percent versus 0 percent, P = 0.002), and sperm abnormalities (48 percent versus 0 percent, P = 0.0001), were statistically higher in SLE patients versus controls. SLE patients with sexual/erectile disfunction had no sexual activity in the last month versus 95 percent of SLE patients without dysfunction (P = 0.0001). On the other hand, no differences were evidenced in SLE patients with or without sexual/erectile disfunction according to demographic data, disease activity, cumulative damage and treatment. CONCLUSION: This is the first study to identify sexual/erectile and gonadal disfunction in male SLE patients. A multidisciplinary approach is essential in order to offer preventive measures for these patients.

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Objetivo: Avaliar a função gonadal de homens com miopatias inflamatórias idiopáticas (MII). Métodos: Vinte e cinco pacientes com MII foram avaliados e comparados com 25 homens saudáveis. Os pacientes foram subdivididos em dois sub-grupos de acordo com as alterações dos espermatozóides: grupo A (n=10, duas ou mais das seguintes alterações: terato/oligo/astenozoospermia ou azoospermia) e grupo B (n=15, teratozoospermia). Foram realizados: exame urológico, ultra-sonografia testicular, análise dos espermatozóides (critérios da OMS e Kruger), pesquisa dos anticorpos anti-espermatozóides e dosagens hormonais Nos subgrupos foram também avaliados: Disease Activity Score (DAS), Visual Analogue Scale (VAS), Manual Muscle Testing (MMT), myositis disease activity assessment visual analogue scales (MYOACT), myositis intention to treat activity index (MITAX), Myositis damage index [MDI], enzimas musculares e tratamento. Resultados: Pacientes apresentaram alterações nos espermatozóides comparados com controles com freqüência maior de nível elevado de FSH (20% versus 0%, p=0,05). O sub-grupo A apresentou freqüência e mediana maior do nível de CK (p=0,001 e p=0,001) assim como DAS (p=0,01), VAS (p=0,051), MMT (p=0,003). As medianas dos volumes testiculares foram menores no grupo A (direito, p=0,015 e esquerdo, p=0,025). As medianas dos parâmetros espermáticos foram reduzidas no grupo A [contagem total (p=0,0001); motilidade (p=0,0001); morfologia pela OMS (p=0,0001) e por Kruger (p=0,0001). A mediana de FSH foi elevada (p=0,035) e de androstenediona foi reduzida (p=0,02) no sub-grupo A. Afrequência de ciclofosfamida foi similar nos grupos (30% versus 6%, p=0,26). Conclusões: Atividade da doença foi o principal fator contribuinte para disfunção gonadal. Hipogonadismo hipergonadotrófico pode explicar as alterações anatômicas e funcionais observadas.

Objective: To perform a global gonad evaluation in male idiopathic inflammatory myopathies (IIM) patients. Methods: Twentyfive consecutive IIM were compared to 25 age-matched healthy subjects. Patients were subdivided in two groups according to the severity of sperm abnormalities: group A (at least two of the following terato/oligo/asthenozoospermia or azoospermia) and group B (teratozoospermia). Patients and controls underwent a systematic assessment consisting of: urologic examination, testicular ultrasound, semen analysis and hormones. Patients´ serum CK levels, visual analogue scale (VAS), disease activity score (DAS), manual muscle testing (MMT), myositis disease activity assessment visual analogue scales (MYOACT), myositis intention to treat activity index (MITAX), and Myositis damage index (MDI) were evaluated. Results: Several sperm variables were significantly altered compared to controls (p<0.05). The subgroup analysis according to the severity of sperm alterations revealed that the frequency of elevated CK and its median level was significantly higher in group A (p=0.001 and p=0.001), as also was DAS, VAS and MMT (p=0.01; p=0.051 and p=0.03). The median of testicular volumes were lower in group A (right p=0.015 and left p=0.025). All median sperm parameters were lower in group A (total sperm count, p=0.0001; total motile sperm count, p=0.0001; and sperm morphology by Kruger p=0.0001 and WHO p=0.0001). Higher median FSH (p=0.035) and lower median androstenedione levels (p=0.02) were observed in group A. The frequency of cyclophosphamide was similar in both groups (30% vs. 6%, p=0.26). Conclusions: Active disease was the major contributing factor for severe gonad dysfunction. The hypergonadotrophic hypogonadism may explain the anatomical and dysfunctional alterations observed.

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The presence of musculoskeletal pain was evaluated in adolescents. Pain was reported by 40% of respondents, benign joint hypermobility syndrome by 10%, myofascial syndrome by 5%, tendonitis by 2%, and fibromialgia by 1%. Logistical regression analysis indicated that sex and age were predictive of pain.

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