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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.

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.

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.

RESUMOAs recomendações propostas pela Comissão de Síndrome de Sjögren da Sociedade Brasileira de Reumatologia para tratamento da síndrome de Sjögren foram baseadas em uma revisão sistemática da literatura nas bases de dados Medline (PubMed) e Cochrane até outubro de 2014 e opinião de especialistas na ausência de artigos sobre o assunto. Foram incluídos 131 artigos classificados de acordo com Oxford & Grade. Essas recomendações foram elaboradas com o objetivo de orientar o manejo adequado e facilitar o acesso aos tratamentos para aqueles pacientes com adequada indicação de recebê-los, considerando o contexto socioeconômico brasileiro e os medicamentos disponíveis no país.

No presente artigo discutiremos como avaliar criticamente as informações obtidas. Para tanto, serão revistos conceitos que a epidemiologia clínica tem colocado à disposição da prática clínica baseada em evidências. A pesquisa clínica busca desenvolver meios diagnósticos e terapêuticos medindo associações, ou relações de causa/efeito, entre um fator em estudo e um desfecho clínico. Onde o fator em estudo é o sintoma, sinal propedêutico, teste laboratorial, exame de imagem, ou tratamento. E o desfecho clínico é o reconhecimento da doença, cura, morte ou limitação. É um erro básico de interpretação tomar os marcadores intermediários como desfechos clínicos. Os desenhos de estudo na área clínica apresentam quatro enfoques principais: diagnóstico, prognóstico, terapêutico, dano/ etiológico. Experimentos animais, estudos anatômicos, fisiológicos, genéticos, farmacológicos, análises econômicas são necessários à formação básica do médico, no entanto, não colaboram diretamente para a tomada de decisão clínica. Os desenhos de estudo são classificados de acordo com a presença de grupo controle, seguimento dos pacientes ao longo do tempo e presença de intervenção dos pesquisadores no sentido de modificar a evolução dos pacientes. A hierarquia da força de evidência científica está fundamentada nestas características e na susceptibilidade aos vícios decorrentes do tipo desenho de estudo. Revisões sistemáticas são consideradas de maior força do que os estudos primários que lhe deram origem, e quando envolvem ensaios clínicos randomizados são colocadas no topo da hierarquia. Desde 1998 é crescente a proporção de "diretrizes clínicas baseadas em evidências" quando comparada às revisões sistemáticas, ou às publicações sobre diretrizes em geral, embora as primeiras sejam ainda em número reduzido. A avaliação crítica do artigo deve responder uma questão clínica, e ser consistente quanto à adequação do desenho de estudo e ao controle dos vícios. Concluímos que se deve oferecer atualização metodológica aos profissionais interessados, e colocar a informação já avaliada à disposição de todos, elaborando e divulgando diretrizes baseadas em evidências.Evidence based health care begins with a clinical question and the search on data bases to retrieve the relevant information, that was the issue of two preceding articles of this series. At present it will be discussed how to critically appraise the medical literature using the clinical epidemiological methodology. Clinical research aims to develop diagnostic and therapeutic procedures measuring association and causality between the exposure and outcome. In this case the exposures are signs, symptoms, laboratorial or image exam, and therapy intervention. It is a mistake to take surrogate end-points instead of clinical outcomes. The main types of clinical study design are diagnostic, prognostic, therapeutic and harm/etiology. Experimental, physiologic and animal studies are useful for the medical undergraduate education, but do not contribute with clinical decisions. The study designs are classified according with the presence of a control group, patient's follow-up, and therapy interventions. The evidence hierarchy was done by the previous characteristics and the presence of systematic bias. Systematic reviews are stronger than the primary observational studies and are on the top when they revised randomized clinical trial. Since 1998 the proportion of evidence based practice guidelines was increasing compared with systematic reviews or other types of practice guidelines, although the former still are in a few numbers. The article critical appraisal must answer the clinical question, and need to have consistent study design and bias under control. In conclusion we ought to offer methodological actualization to interested physicians and put the information already critically assessed on evidence-based practice guidelines.

Abstract Osteoporosis, a metabolic disease characterized by low bone mass, deterioration of the bone tissue microarchitecture and increased susceptibility to fractures, is commonly regarded as a women's health problem. This point of view is based on the fact that compared with men, women have lower bone mineral density and longer lifespans and lose bone mass faster, especially after menopause, due to a marked decrease in serum estrogen levels. However, in the last 20 years, osteoporosis in men has become recognized as a public health problem due to the occurrence of an increasingly higher number of fragility fractures. Approximately 30% of all hip fractures occur in men. Recent studies show that the probability of fracture due to hip, vertebral or wrist fragility in Caucasian men older than fifty years, for the rest of their lives, is approximately 13% versus a 40% probability of fragility fractures in women. Men show bone mass loss and fractures later than women. Although older men have a higher risk of fracture, approximately half of all hip fractures occur before the age of 80. Life expectancy is increasing for both sexes in Brazil and worldwide, albeit at a higher rate for men than for women. This Guideline was based on a systematic review of the literature on the prevalence, etiology, diagnosis and treatment of osteoporosis in men.

Abstract Objective To establish guidelines based on scientific evidence for the diagnosis of fibromyalgia. Material and methods Evidence collection was performed based on 9 questions regarding the diagnosis of fibromyalgia, structured using the Patient, Intervention or Indicator, Comparison and Outcome (P.I.C.O.), with searches in the main, primary databases of scientific information. After defining the potential studies to support the recommendations, they were graded according to evidence and degree of recommendation.

Osteoporosis is the leading cause of fractures in the population older than 50 years. This silent disease affects primarily postmenopausal women and the elderly, and the morbidity and mortality rates are high. The main goal of treating osteoporosis is the prevention of fractures. The identification of populations at risk through early diagnosis and treatment is essential. The last Brazilian guideline for the treatment of postmenopausal osteoporosis was elaborated in 2002. Since then, new strategies for diagnosis and risk stratification have been developed, and drugs with novel action mechanisms have been added to the therapeutic arsenal. The Osteoporosis and Osteometabolic Diseases Committee of the Brazilian Society of Rheumatology, in conjunction with the Brazilian Medical Association and other Societies, has developed this update of the guidelines for the treatment of postmenopausal osteoporosis according to the best scientific evidence available. This update is intended for professionals in many medical and health specialties involved in the treatment of osteoporosis, for physicians in general and for health-related organizations., (Copyright © 2017. Published by Elsevier Editora Ltda.)

The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed), EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease., (Copyright © 2017. Published by Elsevier Editora Ltda.)

Osteoporosis, a metabolic disease characterized by low bone mass, deterioration of the bone tissue microarchitecture and increased susceptibility to fractures, is commonly regarded as a women's health problem. This point of view is based on the fact that compared with men, women have lower bone mineral density and longer lifespans and lose bone mass faster, especially after menopause, due to a marked decrease in serum estrogen levels. However, in the last 20 years, osteoporosis in men has become recognized as a public health problem due to the occurrence of an increasingly higher number of fragility fractures. Approximately 30% of all hip fractures occur in men. Recent studies show that the probability of fracture due to hip, vertebral or wrist fragility in Caucasian men older than fifty years, for the rest of their lives, is approximately 13% versus a 40% probability of fragility fractures in women. Men show bone mass loss and fractures later than women. Although older men have a higher risk of fracture, approximately half of all hip fractures occur before the age of 80. Life expectancy is increasing for both sexes in Brazil and worldwide, albeit at a higher rate for men than for women. This Guideline was based on a systematic review of the literature on the prevalence, etiology, diagnosis and treatment of osteoporosis in men., (Copyright © 2017. Published by Elsevier Editora Ltda.)

Objective: To establish guidelines based on scientific evidence for the diagnosis of fibromyalgia., Material and Methods: Evidence collection was performed based on 9 questions regarding the diagnosis of fibromyalgia, structured using the Patient, Intervention or Indicator, Comparison and Outcome (P.I.C.O.), with searches in the main, primary databases of scientific information. After defining the potential studies to support the recommendations, they were graded according to evidence and degree of recommendation., (Copyright © 2017. Published by Elsevier Editora Ltda.)

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., (Copyright © 2015 Elsevier Editora Ltda. All rights reserved.)

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., (Copyright © 2015 Elsevier Editora Ltda. All rights reserved.)

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., (Copyright © 2015 Elsevier Editora Ltda. All rights reserved.)

The recommendations proposed by the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology for the treatment of Sjögren's syndrome were based on a systematic review of literature in Medline (PubMed) and the Cochrane databases until October 2014 and on expert opinion in the absence of studies on the subject. 131 items classified according to Oxford & Grade were included. These recommendations were developed in order to guide the appropriate management and facilitate the access to treatment for those patients with an appropriate indication, considering the Brazilian socioeconomic context and pharmacological agents available in this country., (Copyright © 2015 Elsevier Editora Ltda. All rights reserved.)