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RESUMO Introdução: O pioderma gangrenoso (PG) é uma doença neutrofílica, rara, porém de consequências danosas. O Capítulo de Feridas da Sociedade Brasileira de Cirurgia Plástica (SBCP) foi instado a compilar as melhores práticas, tanto diagnósticas como terapêuticas, junto às Sociedades Brasileiras de Dermatologia e Reumatologia para um melhor esclarecimento dos seus membros. Métodos: Ampla revisão de artigos publicados na literatura médica e compilação das novas diretrizes de diagnóstico e tratamento por dois membros indicados por cada uma das Sociedades Brasileiras de Cirurgia Plástica, Dermatologia e Reumatologia. Resultados: O PG deixou de ser uma doença de exclusão, tendo os critérios diagnósticos bem definidos e a orientação terapêutica delineada pelos autores, incluindo o uso de terapia biológica. Conclusão: O PG permanece desafiador, mas sistematizar a investigação e o uso dos novos medicamentos, bem como o manejo das feridas, abre novas perspectivas, interferindo na fisiopatologia de modo positivo, com maior precocidade e menos efeitos colaterais do que a terapia imunossupressora de forma isolada. ABSTRACT Introduction: The pyoderma gangrenosum (PG) is a neutrophilic disease, rare but with a poor outcome. The Capitulum of Wound treatment of the Brazilian Society of Plastic Surgery (SBCP) promoted a discussion with the Brazilian societies of Dermatology and Rheumatology to extract the best procedures in diagnostic and treatment. Methods: Broad review of published articles related to the subject and compilation of guidelines of diagnostic and treatment by two advisors of each involved society, plastic surgery, dermatology and rheumatology. Results: The PG is not an exclusion disease anymore, with well defined criteria for its diagnostic and literature based treatment, refined by the authors, including the use of biological therapies. Conclusion: The PG remains challenging, but systematizing the investigation and the use of new drugs has opened a new horizon of treatments, interfering in the pathophysiology in a positive manner with fewer side effects than immunosuppressive therapy alone.

RESUMO: Objetivo: Descrever as características clínicas e imunológicas dos pacientes com esclerose sistêmica em hospital terciário de Goiânia. Métodos: Trata-se de um estudo observacional, descritivo, retrospectivo em que os dados foram coletados dos prontuários dos pacientes atendidos no ambulatório de Reumatologia do Hospital Geral de Goiânia (HGG), durante o período de janeiro de 2006 a dezembro de 2015. Resultados: De acordo com os critérios de inclusão e exclusão, foram incluídos 27 pacientes. Destes, 81,5% pacientes eram mulheres. A idade média foi de 49,7 anos. Em relação às formas da doença, 59,3% dos pacientes apresentavam a forma limitada, 14,8% apresentavam a forma difusa e 22,2% apresentavam a forma localizada. As principais manifestações clínicas observadas foram fenômeno de Raynaud (44,5%) e acometimento esofágico (44,5%). A pneumopatia intersticial esteve presente em 100% dos casos da forma difusa. Conclusões: O perfil clínico e imunológico encontrado no Hospital Geral de Goiânia é semelhante ao encontrado na literatura, com diferenças pontuais. A descrição destas características pode contribuir para definições de ações de cuidado específicas para esse grupo, junto aos órgãos de atenção à saúde pública.

Background/objective The epidemiology of vasculitis is variable in different geographic areas, and this issue has not been approached in Brazil yet. The objective of this study was to assess the frequency of vasculitis in specialized centers in Brazil. Methods This cross-sectional study was performed in 9 vasculitis outpatient clinics from 6 different states mainly from the Southeast and the Northeast regions of Brazil between 2015 and 2017. Diagnosis and/or classification criteria for Behcet disease (BD), Takayasu arteritis (TA), giant cell arteritis (GCA), polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and cryoglobulinemic vasculitis (CryoVas) were used to include patients with at least 6 months of follow-up in this hospital-based survey. Results A total of 1233 patients with systemic vasculitis were included from the Southeast region. Behcet disease was the most frequent vasculitis (35.0%) followed by TA (26.4%), GPA (16.2%), PAN (5.8%), GCA (5.8%), EGPA (4.3%), MPA (3.4%), and CryoVas (3.0%). Up to 7.8% of vasculitis patients had a juvenile onset, and the frequency of vasculitides found in children and adolescents was as follows: TA (52.6%), BD (24.7%), GPA (12.4%), and PAN (10.3%). No cases of EGPA, MPA, and CryoVas were diagnosed before the age of 18 years. As a comparator, 103 vasculitis patients were included in the Northeast of Brazil where TA was found in 36.9% and BD in 31.1% of vasculitis cases. No GCA cases were found in the Northeast part of Brazil. Conclusions Similar to the epidemiology of vasculitis in Asia, BD and TA are the most frequent vasculitis in Southeastern Brazilian referral centers.

SUMMARY The 2006 Revised Sapporo Classification Criteria for Definite Antiphospholipid Syndrome included as laboratory criteria the tests for antiphospholipid antibodies whose accuracy was regarded as satisfactory according to the evidence available at that time. In practice, however, the sensitivity and specificity of these “criteria” of antiphospholipid antibodies are sometimes insufficient for identifying or ruling out antiphospholipid syndrome. It has been studied whether the accuracy of the laboratory diagnosis of the syndrome could be improved by testing for non-criteria antiphospholipid antibodies. In this work, we review evidence on the clinical associations and diagnostic value of the most commonly studied non-criteria antibodies, namely: antiphosphatidylethanolamine, anti-annexin A5, anti-prothrombin, anti-phosphatidylserine/prothrombin complex, IgA anticardiolipin, and IgG anti-domain I of the β2 glycoprotein antibodies. RESUMO A classificação de Sapporo revisada para a síndrome antifosfolipídica definida de 2006 incluiu como critérios laboratoriais aqueles testes para anticorpos antifosfolípides cuja acurácia era considerada satisfatória de acordo com a evidência então disponível. Porém, na prática, a sensibilidade e especificidade desses anticorpos antifosfolípides “critério” são por vezes insuficientes para identificar ou descartar a síndrome antifosfolípide. Tem-se estudado se a acurácia do diagnóstico laboratorial da síndrome poderia ser melhorada por meio da testagem de anticorpos antifosfolípides não critério. Neste trabalho revisamos a evidência a respeito das associações clínicas e valor diagnóstico dos anticorpos não critério mais estudados, nomeadamente: anticorpos antifosfatidiletanolamina, antianexina A5, antiprotrombina, anticomplexo fosfatidilserina/protrombina, IgA anticardiolipina e IgG antidomínio I da anti-β2 glicoproteína I.

Summary Antiphospholipid syndrome (APS) is an autoimmune disease characterized by antiphospholipid antibodies (aPL) associated with thrombosis and/or pregnancy morbidity. Most APS events are directly related to thrombotic events, which may affect small, medium or large vessels. Other clinical features like thrombocytopenia, nephropathy, cardiac valve disease, cognitive dysfunction and skin ulcers (called non-criteria manifestations) add significant morbidity to this syndrome and represent clinical situations that are challenging. APS was initially described in patients with systemic lupus erythematosus (SLE) but it can occur in patients without any other autoimmune disease. Despite the autoimmune nature of this syndrome, APS treatment is still based on anticoagulation and antiplatelet therapy. Resumo A síndrome antifosfolipídide (APS) é uma doença autoimune caracterizada por tromboses e morbidade gestacional associadas à positividade de antiphospholipid antibodies (aPL). A maioria das manifestações da APS está diretamente relacionada aos eventos trombóticos, que podem afetar pequenos, médios ou grandes vasos. Outras manifestações como trombocitopenia, nefropatia, valvulopatia, disfunção cognitiva e úlceras cutâneas (chamadas de manifestações não critérios) agregam significativa morbidade e muitas vezes são refratárias ao tratamento convencional. Embora tenha sido inicialmente descrita em pacientes com lúpus eritematoso sistêmico (LES), a síndrome antifosfolípide também pode ocorrer em pacientes sem outras doenças autoimunes associadas. Apesar do caráter autoimune dessa síndrome, o tratamento da APS ainda é baseado na anticoagulação e na antiagregação plaquetária.

After publication of the original article [1], we were notified that there is a mistake in Fig. 2.

Background The term Direct Oral Anticoagulants (DOACs) refers to a group of drugs that inhibit factor Xa or thrombin. Even though their use for treating different thrombotic or prothrombotic conditions is increasing recently, there is no compelling evidence indicating that those medications are safe in all antiphospholipid syndrome (APS) patients. Methodology To address this issue, specialists from the Antiphospholipid Syndrome Committee of the Brazilian Society of Rheumatology performed a comprehensive review of the literature regarding DOACs use in APS to answer the three following questions: (1) potential mechanisms of action of these drugs that could be relevant to APS pathogenesis, (2) DOACs interference on lupus anticoagulant testing, and (3) the efficacy of DOACs in APS. Position statement After critically reviewing the relevant evidence, the authors formulated 8 Position Statements about DOACs use in APS. Conclusion DOACs should not be routinely used in APS patients, especially in those with a high-risk profile (triple positivity to aPL, arterial thrombosis, and recurrent thrombotic events). In addition, DOACs interferes with LA testing, leading to false-positive results in patients investigating APS.

Background: The V Brazilian Consensus for determination of autoantibodies against cellular constituents on HEp-2 cells, held in Brasilia (DF, Brazil) on August 27, 2016, discussed the harmonization between the Brazilian Consensus on ANA (BCA) guidelines and the International Consensus on ANA Patterns (ICAP) recommendations (www.anapatterns.org). Initial guidelines were formulated by the group of Brazilian experts with the purpose of guiding and enabling Brazilian clinical laboratories to adopt recommendations and to provide a common standard for national and international consensuses. Mainbody: Twenty Brazilian researchers and experts from universities and clinical laboratories representing the various geographical regions of the country participated in the meeting. Three main topics were discussed, namely the harmonization between the BCA guidelines and latest recommendations of the ICAP initiative, the adjustment of the terminology and report on HEp-2 patterns, and a reassessment of quality assurance parameters. For the three topics, our aim was to establish specific guidelines. All recommendations were based on consensus among participants. There was concrete progress in the adjustment of the BCA guidelines to match the ICAP guidelines. To a certain extent, this derives from the fact that ICAP recommendations were largely based on the algorithm and recommendations of the IV Brazilian ANA Consensus, as consistently recognized in the ICAP publications and presentations. However, although there is great overlap between the two Consensuses, there are some point divergences. These specific items were individually and extensively discussed, and it was acknowledged that in several points ICAP improved recommendations previously issued by the Brazilian ANA Consensus and these changes were readily implemented. Regarding some specific topics, the BCA panel of experts felt that the previously issued recommendations remained relevant and possibly will require further discussion with ICAP. The term anti-cell antibodies was adopted as the recommended designation, recognizing that the assay addresses antibodies against antigens in the nucleus and in other cell compartments. However, the acronym ANA HEp-2 was maintained due to historical and regulatory reasons. It was also signalized that the latest trend in ICAP is to adopt the term Indirect Immunofluorescent Assay on HEp-2 cell substrate (HEp-2 IIFA). In addition, the quality assurance strategies previously presented were ratified and emphasized. Conclusion: The V BCA edition was successful in establishing an overall harmonization with the ICAP recommendations for interpretation of the HEp-2 IIFA test, pinpointing the perspectives in filling the remaining gaps between both initiatives.

Background Moderate exercise improves the functions of the immune system. The results of serum immunoglobulins in response to acute physical exercise are conflicting, but in general, there is an increase in serum immunoglobulins in athletes and non-athletes after maximal and submaximal exercise. The effects of acute physical exercise on serum immunoglobulin in patients with systemic lupus erythematosus are unknown. Objectives To determine the serum levels of IgG, IgM and IgA immunoglobulins in patients with systemic lupus erythematosus after a single session of acute aerobic exercise. Methods Thirty-one women with SLE (ACR1997 and/or SLICC 2012 criteria),11 with active lupus and 20 with inactive lupus, and 24 healthy women, who were matched by gender, age, and body mass index, participated in this study. All subjects underwent a maximal exercise test on motorized treadmill, with increases in velocity every minute until exhaustion, to evaluate the speed (km/h), heart rate, Borg scale (6-20), and the reason for stopping the test (fatigue and/or dyspnea). Samples of blood were collected in the morning, after an 8-hour fast and immediately after acute physical activity, and stored in a freezer at -80°C until the tests were performed. Immunoglobulin IgG, IgM and IgA assay was performed by turbitometry according to the manufacturer (Turbiquest, Labtest Diagnostic S.A). Results There was an increase in both IgG and IgA serum levels after physical activity in SLE patients (p = 0.004 for IgG and p = 0.002 for IgA) and healthy controls (p Conclusion Patients with SLE, mainly with inactive disease, responded to acute physical exercise with increased IgG and IgA serum levels, similarly to healthy controls. References [1] Nehlsen-Cannarella SL, et al. The effects of acute moderate exercise on lymphocyte function and serum immunoglobulin levels. Int J Sports Med. 1991; 12(4): 391-398.. [2] Nieman DC and Nehlsen-Cannarella SL.The effects of acute and chronic exercise of immunoglobulins. Sports Med. 1991; 11(3): 183-201. [3] Ochoa-Martinez PY, et al. Cinetica de la inmunoglobulina-A salival (IgAs) en adultos jovenes con capacidad aerobica promedio o excelente antes y despues de una prueba de esfuerzo maximo. Revista Chilena de Infectologia, 2017; 34: 27-32. Disclosure of Interests None declared

Resumo O objetivo destas recomendacoes e orientar o tratamento apropriado de inducao em pacientes com vasculite associada a anticorpos anticitoplasma de neutrofilos (VAA) ativa. As recomendacoes propostas pelo Comite de Vasculopatias da Sociedade Brasileira de Reumatologia para a terapia de inducao para vasculites associadas aos anticorpos anticitoplasma de neutrofilos (VAA), inclusive granulomatose com poliangiite, poliangiite microscopica e vasculite limitada ao rim, foram baseadas em uma revisao sistematica da literatura e na opiniao de especialistas. A revisao da literatura foi feita com as bases de dados Medline (PubMed), Embase e Cochrane para consultar artigos ate outubro de 2016. As diretrizes Prisma ( Preferred Reporting Items for Systematic Reviews and Meta‐Analyses – Principais itens para reportar revisoes sistematicas e metanalises) foram usadas para a revisao sistematica e os artigos foram avaliados de acordo com os niveis de evidencia Oxford. Dezesseis recomendacoes foram feitas em relacao a diferentes aspectos da terapia de inducao para VAA. O objetivo dessas recomendacoes e servir como um guia para decisoes terapeuticas por profissionais da saude no tratamento de pacientes com VAA que apresentem a doenca ativa.

Resumo Objetivos: Investigar a associação das comorbidades com a limitação da mobilidade e com a incapacidade funcional em pacientes com artrite reumatoide (AR), bem como identificar o indicador de comorbidade mais apropriado para determinar essa associação. Métodos: Em um estudo transversal foram incluídos 60 pacientes com AR por um período de 11 meses. Comorbidades foram avaliadas por meio de três indicadores: (i) número total de comorbidades (NCom); (ii) índice de comorbidade de Charlson (ICC); e (iii) índice de comorbidade funcional (ICF). A atividade da doença foi avaliada pelo Índice de Atividade da Doença 28 (DAS-28/VHS). A capacidade funcional foi mensurada pelo Questionário de Avaliação da Saúde (HAQ) e a mobilidade foi mensurada pelos testes senta-levanta da cadeira cinco vezes (TSL) e timed get up and go (TUG). A análise estatística foi feita por meio de regressão múltipla log-linear Stepwise com nível de significância de 5%. Resultados: No modelo final, apenas o fator comorbidades (ICF) esteve associado à mobilidade (TSL e TUG). O escore no ICF explicou 19,1% da variabilidade do TSL (coeficiente de determinação [R2] = 0,191) e 19,5% da variabilidade do TUG (R2 = 0,195). Em relação à incapacidade funcional (HAQ), os fatores associados foram o fator comorbidades (ICF) e a atividade da doença (DAS-28/VHS) que em conjunto explicaram 32,9% da variabilidade do escore do HAQ (R2 ajustado = 0,329). Conclusão: As comorbidades estão associadas com a limitação da mobilidade e a incapacidade funcional em pacientes com AR. O ICF demonstrou ser um indicador de comorbidade apropriado para determinar essa associação. Abstract Objectives: To investigate the association of comorbidities with mobility limitation and functional disability in patients with rheumatoid arthritis (RA) and to identify which comorbidity indicator is the most appropriate to determine this association. Methods: Sixty RA patients were enrolled in a cross-sectional study for a period of 11 months. Comorbidities were assessed using three indicators: (i) the total number of comorbidities (NCom); (ii) the Charlson comorbidity index (CCI); and (iii) the functional comorbidity index (FCI). Disease activity was assessed using the Disease Activity Score 28 (DAS-28/ESR). Functional capacity was measured using the Health Assessment Questionnaire (HAQ), and mobility was measured using Timed Up and Go Test (TUG) and Five Times Sit To Stand Test (FTSTS). Statistical analysis was performed using a stepwise log-linear multiple regression with a significance level of 5%. Results: In the final model, only comorbidity (FCI) was associated with mobility limitation (FTSTS and TUG). The FCI score explained 19.1% of the variability of the FTSTS (coefficient of determination [R2] = 0.191) and 19.5% of the TUG variability (R2 = 0.195). With regard to functional disability (HAQ), the associated factors were comorbidity (FCI) and disease activity (DAS-28/ESR), which together explained 32.9% of the variability of the HAQ score (adjusted R2 = 0.329). Conclusion: Comorbidities were associated with mobility limitation and functional disability in RA patients. The FCI proved to be an appropriate comorbidity indicator to determine this association.

ResumoObjetivosIdentificar a prevalência de quedas nos últimos 12 meses em pacientes com artrite reumatoide (AR) e verificar a influência da atividade da doença e da capacidade funcional no risco de quedas.Pacientes e métodosParticiparam do estudo 43 pacientes com AR. Foram avaliados os seguintes parâmetros: aspectos clínicos; ocorrência de quedas nos últimos 12 meses; VHS (mm/h); dor, através da escala visual analógica (EVA) com escore de 0 a 10 cm; atividade da doença, medida pelo Índice de Atividade da Doença – 28/VHS (Disease Activity Score 28 – DAS‐28/VHS); capacidade funcional, avaliada pelo Questionário de Avaliação da Saúde (Health Assessment Questionnaire – HAQ); e o risco de quedas, avaliado por meio de dois testes, o teste senta‐levanta da cadeira cinco vezes (TSL) e o teste timed get up and go (TUG).ResultadosA prevalência de quedas nos últimos 12 meses foi de 30,2% (13/43). O fator independente que influenciou significativamente o desempenho no TSL foi o escore total do HAQ, sendo que as demais variáveis não conseguiram contribuir de forma significativa na explicação da variabilidade no TSL. A variável HAQ foi responsável por explicar 42,9% (P

OBJECTIVE: The Third Brazilian Consensus for autoantibodies Screening in HEp-2 cells had as purpose the evaluation of difficulties in the accomplishment of the 2nd Consensus recommendations that took place in the year of 2002, the discussion of strategies for quality control of the assay and the promotion of an update of the clinical associations of the several immunofluorescent patterns. METHODS:Several ANA experts from university centers and private laboratories in different areas in Brazil joined the workshop in Goiânia on 2008 April 13 and 14 with the purpose of discussing and approving the recommendations for standardization, interpretation and use of the test by physicians. Commercial representatives of different ANA slide brands were also invited as listeners to the workshop. RESULTS AND CONCLUSIONS: The 3rd Consensus emphasized the need for quality control in indirect immunofluorescent since there is a considerable heterogeneity of available microscopes and reagents. It also promoted adaptations in the previously approved terminology used to classify the different patterns and finally updated the clinical associations of the several patterns with the purpose of providing guidance for interpretation of the assay by clinical pathologists and assistant physicians.

Resumo Objetivo: Apresentar um caso de sindrome nefrotica, secundaria a sifilis, mimetizando Lupus Eritematoso Sistemico. Relato do caso: Mulher jovem, de 26 anos, evoluiu com ulceras orais dolorosas, artralgia e sindrome nefrotica, sendo aventada, inicialmente, a possibilidade de doenca autoimune. Os resultados dos exames laboratoriais afastaram a possibilidade de lupus eritematoso sistemico e confirmaram o diagnostico de sifilis. Consideracoes finais: A presenca de sindrome nefrotica, em paciente jovem do genero feminino, deve suscitar a investigacao da historia sexual pregressa, sendo a sifilis uma possibilidade obrigatoria nesta investigacao, a qual pode mimetizar o LES, retardando o diagnostico precoce e o tratamento eficaz. Palavras-chave: Lupus eritematoso sistemico. Sindrome nefrotica. Sifilis Abstract Objective: To present a case of nephrotic syndrome, secondary to syphilis, mimicking systemic lupus erythematosus. Case report: 26-year-old woman who developed painful oral ulcers, arthralgia and nephrotic syndrome, and the possibility of autoimmune disease was initially suggested. The results of the laboratory tests removed the possibility of systemic lupus erythematosus and confirmed the diagnosis of syphilis. Final considerations: The presence of nephrotic syndrome in a young female patient should trigger the investigation of previous sexual history, and syphilis is a mandatory possibility in this investigation, which can mimic SLE, delaying early diagnosis and effective treatment. Keyword: Systemic lupus erythematosus. Nephrotic syndrome. Syphilis.

Resumo Objetivo: Descrever um caso clinico de uma mulher de 46 anos, com superposicao de esclerose sistemica e artrite reumatoide, que desenvolve paniculite septal e amiloidose. Relato do caso: Paciente do genero feminino, 46 anos, com diagnostico de sindrome de superposicao (esclerose sistemica e artrite reumatoide) ha oito anos, evolui com dor e aumento de volume das pernas. Aventada a hipotese de paniculite, a paciente foi medicada com anti-inflamatorios, nao obtendo melhora. Submetida a biopsia de pele da perna direita, o quadro histopatologico foi compativel com amiloidose e discreta paniculite septal. Consideracoes finais: A presenca de paniculite, de dificil resposta ao tratamento antiinflamatorio, deve suscitar a pesquisa de doencas infiltrativas, tais como a amiloidose. A presenca de deposito amiloide na derme implica na investigacao de doenca sistemica. Palavras-chave: Esclerose sistemica. Artrite reumatoide. Paniculite. Amiloidose. Abstract Objective: To describe a clinical case of a 46-year-old woman, with overlap of systemic sclerosis and rheumatoid arthritis who develop panniculitis septal and amyloidosis. Case report: A 46-year-old female patient with a diagnosis of overlap syndrome (systemic sclerosis and rheumatoid arthritis) eight years ago evolves with pain and enlargement of the legs . After the hypothesis of panniculitis, the patient was medicated with antiinflammatory drugs and did not obtain improvement. Subjected to skin biopsy of the right leg, histopathological findings were compatible with amyloidosis and discrete panniculitis septal. Final considerations: The presence of panniculitis, which is difficult to respond to antiinflammatory treatment, should lead to the investigation of infiltrative diseases, such as amyloidosis. The presence of amyloid deposits in the dermis implies the investigation of systemic disease. Keyword: Systemic sclerosis. Rheumatoid arthritis. Panniculitis. Amyloidosis.

Objetivo: Determinar a frequência de anticorpos antiparvovírus B19 (B19) em pacientes com artrite reumatoide (AR) e lúpus eritematoso sistêmico (LES), e a possível correlação da soropositividade anti-B19 com a atividade das doenças e a qualidade de vida. Pacientes e métodos: Foram utilizadas amostras séricas de 57 pacientes com AR, 45 com LES e 65 controles sadios. Empregou-se protocolo com dados clínicos, os índices Disease Activity Score 28 (DAS 28), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) e Health Assessment Questionnaire (HAQ). Realizou-se a sorologia anti-B19 por ensaio imunoenzimático (ELISA). Resultados: A média de idade dos pacientes foi de 42,74 14,09 anos, e a dos controles foi de 38,38 13,42 anos.Tinham doença ativa 79 (77,5%) pacientes, e doença inativa 23 (22,5%).Anti-B19 (IgG) foi reagente em 49 (86,0%) IC 95% (77,0 - 95,0)% pacientes com AR, em 38 (84,4%) IC 95% (73,9 - 95,0)% com LES e em 40 (61,5%) IC 95% (49,7 - 73,4)% controles (p = 0,002). Anti-B19 (IgM) foi reagente em 3 (5,3%) IC 95% (0,0 - 11,1)% pacientes com AR, em 7 (15,6%) IC 95% (5,0 - 26,2)% pacientes com LES e em 1 (1,5%) IC 95% (0,0 - 4,5)% controle (p = 0,011). Não houve correlação da reatividade anti-B19 com a atividade das doenças, os índices DAS 28, SLEDAI e HAQ. Conclusão: O presente estudo demonstrou que a população avaliada está exposta à infecção pelo B19, o que demanda atenção com suas manifestações, principalmente entre os pacientes que apresentam maior risco, como os imunossuprimidos.

A síndrome do anticorpo antifosfolipídeo (SAF) é uma doença sistêmica autoimune caracterizada por trombose arterial e venosa, morbidade gestacional e presença de níveis séricos de anticorpos antifosfolipídeos elevados e persistentemente positivos. O tratamento da SAF ainda é sujeito a controvérsias, já que qualquer decisão terapêutica potencialmente irá confrontar-se com o risco de uma cobertura antitrombótica insuficiente ou com o risco excessivo associado à anticoagulação e seus principais efeitos adversos. Esta diretriz foi elaborada a partir de nove questões clínicas relevantes e relacionadas ao tratamento da SAF pela Comissão de Vasculopatias da Sociedade Brasileira de Reumatologia. O objetivo deste trabalho foi criar uma diretriz que incluísse as questões mais relevantes e controversas no tratamento da SAF, com base na melhor evidência científica disponível. As questões foram estruturadas por meio do P.I.C.O. (paciente, intervenção ou indicador, comparação e outcome/desfecho), o que possibilitou a geração de estratégias de busca da evidência nas principais bases primárias de informação científica (MEDLINE/Pubmed, Embase, Lilacs/Scielo, Cochrane Library, Premedline via OVID). Também realizou-se busca manual da evidência e de teses (BDTD e IBICT). A evidência recuperada foi selecionada a partir da avaliação crítica, utilizando instrumentos (escores) discriminatórios de acordo com a categoria da questão terapêutica (JADAD para ensaios clínicos randomizados e New Castle Ottawa Scale para estudos não randomizados). Após definir os estudos potenciais para sustento das recomendações, eles foram selecionados pela força da evidência e pelo grau de recomendação, segundo a classificação de Oxford. The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial and venous thrombosis, gestational morbidity and presence of elevated and persistently positive serum titers of antiphospholipid antibodies. The treatment of APS is still controversial, because any therapeutic decision potentially faces the risk of an insufficient or excessive antithrombotic coverage associated with anticoagulation and its major adverse effects. This guideline was elaborated from nine relevant clinical questions related to the treatment of APS by the Committee of Vasculopathies of the Brazilian Society of Rheumatology. Thus, this study aimed at establishing a guideline that included the most relevant and controversial questions in APS treatment, based on the best scientific evidence available. The questions were structured by use of the PICO (patient, intervention or indicator, comparison and outcome) process, enabling the generation of search strategies for evidence in the major primary scientific databases (MEDLINE/PubMed, Embase, Lilacs, Scielo, Cochrane Library, Premedline via OVID). A manual search for evidence and theses was also conducted (BDTD and IBICT). The evidence retrieved was selected based on critical assessment by using discriminatory instruments (scores) according to the category of the therapeutic question (JADAD scale for randomized clinical trials and Newcastle-Ottawa scale for non-randomized studies). After defining the potential studies to support the recommendations, they were selected according to level of evidence and grade of recommendation, according to the Oxford classification.

In recent years, mediators synthesized in the adipose tissue, the so-called adipokines, have been described. They have a hormonal action, regulating appetite and glucose metabolism, but also act as cytokines with effects on the immune system, including effects on autoimmunity. The most important adipokines are leptin, adiponectin, resistin and visfatin, and some of them have been assessed in autoimmune rheumatic diseases, especially systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). Studies have shown high levels of leptin and adiponectin in SLE, but correlation with disease activity is questionable. In RA, studies have also reported increased levels of leptin and adiponectin, and correlation with disease activity and joint erosion, but the results are conflicting. This review describes the role of leptin and adiponectin on the immune system, as well as on SLE and RA.

OBJETIVO: O III Consenso Brasileiro para Pesquisa de Autoanticorpos em Células HEp-2 (FAN) objetivou discutir estratégias para controlar a qualidade do ensaio, promover a atualização das associações clínicas dos diversos padrões e avaliar as dificuldades de implantação do II Consenso ocorrido no ano de 2002. MÉTODOS: Nos dias 13 e 14 de abril de 2007 participaram do encontro em Goiânia pesquisadores e especialistas de diversos centros universitários e laboratórios clínicos de diferentes regiões do Brasil, com o propósito de discutir e aprovar as recomendações que visam a melhores padronização, interpretação e utilização do ensaio pelos clínicos. Foram convidados como ouvintes representantes comerciais de diferentes empresas produtoras de insumos para realização do teste de FAN. RESULTADOS E CONCLUSÃO: Dada a heterogeneidade de microscópios e reagentes disponíveis no mercado, o III Consenso enfatizou a necessidade do controle de qualidade em ensaios de imunofluorescência indireta. Foram também feitas algumas adequações na terminologia utilizada para classificar os diferentes padrões. Finalmente, foi realizada uma atualização das associações clínicas com finalidade de facilitar cada vez mais o melhor uso do ensaio pelos clínicos. OBJECTIVE: The Third Brazilian Consensus for Autoantibodies Screening in HEp-2 Cells (ANA) had as purpose the evaluation of difficulties in the accomplishment of the 2nd Consensus recommendations that took place in the year of 2002, the discussion of strategies for quality control of the assay and the discussion of an update of the clinical associations of the several immunofluorescent patterns. METHODS: Several ANA experts from university centers and private laboratories in different areas in Brazil joined the workshop in Goiânia on 2007 April 13 and 14 with the purpose of discussing and approving the recommendations for standardization, interpretation and use of the test by physicians. Commercial representatives of different ANA slide brands were also invited as listeners to the workshop. RESULTS AND CONCLUSION: The 3rd ANA Consensus emphasized the need for quality control in indirect immunofluorescent assays since there is a considerable heterogeneity of available microscopes and reagents. It also promoted adaptations in the previously approved terminology used to classify the different patterns and finally updated the clinical associations of the several patterns with the purpose of providing guidance for interpretation of the assay by clinical pathologists and assistant physicians. UCG Universidade Federal de São Paulo (UNIFESP) UNIFESP Programa de Pós-Graduação da disciplina de Reumatologia Fleury Medicina e Saúde setor de Imunologia UCG Departamento de Biomedicina Laboratório de Apoio Didático Centro Imuno-Reumatológico de São Paulo AFIP-Medicina Laboratorial Padrão Laboratório Clínico Universidade Federal de Uberlândia UFU Hospital das Clínicas serviço de reumatologia Pontifícia Universidade do Rio Grande do Sul Faculdade de Medicina Metanalysis Centro de Diagnósticos Médicos Universidade Federal do Pará Laboratório Amaral Costa Centro de Educação Técnica do Estado do Pará NewLife Comércio de Produtos Laboratoriais Universidade de São Paulo Faculdade de Medicina Hospital das Clínicas FMUSP Hospital das Clínicas Divisão de Laboratório Central Hospital Israelita Albert Einstein Departamento de Patologia Clínica Instituto Hermes Pardini Biometrix Diagnóstica FMUSP Laboratório Atalaia Sérgio Franco Medicina Diagnóstica setor de Imunoensaios UCG Faculdade de Medicina Hospital das Clínicas Universidade do Sul de Santa Catarina Universidade do Vale do Itajaí Laboratório Médico Santa Luzia Hospital e Maternidade e Jardim América Laboratórios Saúde Hemagen Diagnósticos Universidade Federal da Bahia Fundação Bahiana para Desenvolvimento das Ciências Hospital Santa Izabel serviço de reumatologia Universidade Federal de Mato Grosso FMUCG Alka Universidade do Estado do Rio de Janeiro Hospital Pró-cardíaco Centro de Autoimunidades Diagnósticos da América SA Laboratório Diagnósticos da América SA setor de Imunologia Universidade Federal de Minas Gerais Faculdade de Medicina UFMG Hospital das Clínicas Serviço de Medicina Laboratorial Hospital Universitário de Brasília Laboratório de Reumatologia União Educacional do Planalto Central Centro Universitário Euro-Americano Imunotech Sistemas Diagnósticos Importação e Exportação UNIFESP, Programa de Pós-Graduação da disciplina de Reumatologia SciELO

OBJETIVO: O Segundo Consenso Brasileiro de Fator Antinuclear (FAN) em Células HEp-2 ratificou os algoritmos de decisão para leitura dos padrões do FAN na imunofluorescência indireta vistos na primeira edição do Consenso Brasileiro, adicionando ainda um novo algoritmo relacionado com os padrões mistos. MÉTODOS: Tendo em vista a habilidade do teste em detectar autoantígenos nos distintos compartimentos celulares, e não apenas no núcleo, propõe-se novas denominações para este exame laboratorial. RESULTADOS E CONCLUSÕES: Como novas denominações algumas sugestões foram igualmente aceitas, dentro do tema pesquisa de auto-anticorpos contra constituintes do núcleo (FAN HEp-2), nucléolo, citoplasma e aparelho mitótico. Foram abordadas as principais relevâncias clínicas com os padrões de FAN descritos, facilitando o melhor uso do ensaio pelo médico. OBJECTIVE: The Second Brazilian Consensus on Antinuclear Antibodies (ANA) in HEp-2 Cells approved and extended the decision trees developed during the First Brazilian Consensus in order to also offer information about mixed patterns of fluorescence. METHODS: Since this test elicits reactions not only to nuclear autoimmune antigens but also to different cell compartments, new denominations for the test were approved. Results and CONCLUSIONS: These new denominations encompass variations on the autoantibody testing against the nucleus (ANA HEp-2), nucleolus, cytoplasm, and mitotic apparatus issue. Furthermore, major clinical associations were described for each immunofluorescent pattern, facilitating the interpretation of laboratory results in the clinical practice. FMUSP UNIFESP Bio-Rad Laboratório Brasil Hospital Geral de Goiânia Biomédica Universidade Federal de Uberlândia UFMG HC PUCRS HC New Life Produtos Hospitalares Universidade Católica de Goiás FMUSP HC Laboratório Central Patologista Clínica FMUSP HC Frischmann Aisengart Unidad Inmunología Universidade Católica de Goiás Laboratório de Auto-Imunidade Exame Medicina Laboratorial FMUFG HC Laboratório de Imuno-Reumatologia e HLA Lab. Santa Luzia Medivax/Bion HSPE/SP Universidade Católica de Goiás Laboratório da Área de Saúde Farmacêutica-Bioquímica Univ. Fed. Mato Grosso FMUFG Serviço de Reumatologia Hospital Durand Unidad Inmunología Laboratório Clínico UFRGS HCPA Serviço de Reumatologia UERJ FCM UFMG FM Hospital Universitário de Brasília Laboratório de Reumatologia UNIFESP SciELO

A análise da presença de auto-anticorpos feita por imunofluorescência indireta em células HEp-2 constitui-se em um método de triagem escolhido na maioria dos laboratórios clínicos. A ausência de uma nomenclatura definida para a descrição dos laudos tem trazido problemas na utilização clínica do teste, pelas dificuldades no controle de qualidade e na padronização dos resultados, que, por sua vez, embora similares, recebiam denominações diferentes. O I Consenso Brasileiro para Padronização dos Laudos de FAN HEp-2 reuniu em agosto de 2000, em Goiânia, diversos especialistas de todo o Brasil. Esses emitiram pareceres em consenso para os distintos padrões: nucleares, nucleolares, citoplasmáticos e aparelho mitótico. Foram feitas recomendações sobre os critérios para a leitura de uma lâmina, bem como para relação entre a diluição de triagem e o sistema óptico utilizado. The technique of immunofluorescence using HEp-2 cells as substrate is the screening method of choice for the presence of autoantibodies in many clinical laboratories. The lack of a specific terminology for reporting results brings problems in quality control, clinical utility of the test, and standardization attempts. The first Brazilian Consensus for Standardization of ANA in HEp-2 Cells took place in Goiânia in August 2000. Several laboratory specialists with experience in the methodology showed up. They established guidelines for the description of ANA patterns in the Portuguese language, encompassing distinct descriptions for nuclear, nucleolar, cytoplasmic and mitotic apparatus patterns of fluorescence. Recommendations were also established regarding screening titers, final dilution titer, and on morphological criteria for reading the slides.

Background Comorbidities in rheumatoid arthritis (RA) are associated with different outcomes. 1 Although some studies have shown associations between such comorbidities and functional incapacity, studies are needed to assess their influence on the mobility of individuals with RA. 2,3 Objectives To establish the prevalence of comorbidities in RA patients and to assess their influence on the functional capacity and mobility of the affected individuals. Methods In a cross-sectional study we included 60 patients with RA fulfilling the American College of Rheumatology criteria (ACR, 1987) over a period of 11 months. Comorbidities were assessed by means of three indicators: (i) total number of comorbidities; (ii) the Charlson Comorbidity Index (CCI) 4 ; and (iii) the Functional Comorbidity Index (FCI) 5 . The activity of disease was evaluated by the Disease Activity Score 28 (DAS28/ESR). The participants9 functional capacity was measured using the Health Assessment Questionnaire (HAQ), and their mobility was measured using the Five-Times-Sit-to-Stand (FTSTS) 6 and Timed Get Up and Go (TUG) 7 tests. Statistical analysis was performed using log-linear stepwise multiple regression at 5% significance level. Results The prevalence of comorbidities in the investigated sample of patients with RA was 90% when the total number of comorbidities was taken into consideration. In the final multiple regression model, the independent factors that influenced functional capacity (HAQ) were activity of disease (DAS28/ESR) and comorbidities, as assessed by FCI. The adjusted R 2 of these factors taken as a whole was 0.329. With respect to the participants9 mobility (FTSTS and TUG), only the independent factor comorbidities (FCI) exerted a significant influence on the results. The FCI scores explained 19.1% of the FTSTS variability (R 2 =0.191) and 19.5% of the TUG variability (R 2 =0.195). Conclusions Comorbidities were highly prevalent in individuals with RA and exerted a negative influence on their functional capacity and mobility. FCI proved to be satisfactory to assess the association between comorbidities and physical function in individuals with RA. References Gullick NJ, Scott DL. Co-morbidities in established rheumatoid arthritis. Best Pract Res Clin Rheumatol 2011; 25:469-483. Radner H, Smolen JS, Aletaha D. Impact of comorbidity on physical function in patients with rheumatoid arthritis. Ann Rheum Dis 2010; 69:536-541. Norton S, Koduri G, Nikiphorou E, et al. A study of baseline prevalence and cumulative incidence of comorbidity and extra-articular manifestations in RA and their impact on outcome. Rheumatology (Oxford) 2013; 52:99-110. Charlson ME, Pompei P, Ales KL, et al. A new method of classifying prognostic comorbidity in longitudinal studies: development and validation. J Chronic Dis 1987;40:373-383. Groll DL, To T, Bombardier C, et al. The development of a comorbidity index with physical function as the outcome. J Clin Epidemiol 2005;25:595-602. Bohannon RW. Sit-to-stand test for measuring performance of lower extremity muscles. Percept Mot Skills 1995;80:163-6. Podsiadlo D, Richardson S. The time “Up & Go”: a test of basic functional mobility for frail elderly persons. J Am Geriatr Soc 1991;39:142-148. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1924

Albert Einstein Medicina Diagnostica Alka Tecnologia Amaral Costa Laboratorio Conselho Federal de Biomedicina DASA Euroimmun Brasil Grupo Fleury Hemagen Medivax Olimpus Padrao Laboratorio Clinic Pontificia Universidade Catolica de Goias - PUC-Goias Sociedade Brasileira de Patologia Clinica e Medicina Laboratorial Sociedade Brasileira de Reumatologia Thermo Scientific Wama Diagnostica Werfen Group - Werfen Medical Hermes Pardini Conselho Regional de Biomedicina - 3a Regiao Objetivo: O IV Consenso Brasileiro para Pesquisa de Autoanticorpos em Células HEp-2 (FAN) realizado em Vitória (ES), no dia 18 de setembro de 2012, objetivou discutir estratégias e recomendações relacionadas ao procedimento técnico, à padronização e à interpretação dos resultados da pesquisa de autoanticorpos em células HEp-2. Métodos: Participaram do evento 23 pesquisadores e especialistas de Universidades e laboratórios brasileiros. Foram abordados diferentes tópicos, discutidos amplamente a fim de se estabelecer recomendações específicas. Resultados e conclusão: O IV Consenso integrou à árvore de decisão o padrão citoplasmático em Anéis e Bastões, o padrão nuclear pontilhado Quasi-homogêneo (QH) e o padrão misto CENP-F. Discutiu-se ainda a necessidade de atenção para a classificação do padrão misto relacionado à presença de anticorpos anti-DNA topoisomerase I (Scl-70), compreendendo os componentes nuclear pontilhado fino, nucleolar homogêneo, NOR na placa metafásica e citoplasmático pontilhado fino. Foram sugeridas diretrizes para o controle de qualidade do teste, diluição de triagem e diluição de esgotamento, e foi emitido alerta quanto à necessidade de atenção em relação à heterogeneidade de substratos disponíveis no mercado e a utilização de metodologias automatizadas para detecção de autoanticorpos. Objective: the Fourth Brazilian Consensus for Autoantibodies Screening in HEp-2 Cells (ANA) was held in Vitoria, Espirito Santo, and aimed to discuss strategies and recommendations about the technique, standardization, interpretation and quality control of the indirect immunofluorescence reaction on HEp-2 cells.Methods: Twenty three ANA experts from university centers and private laboratories in different areas from Brazil discussed and agreed upon recommendations for the fourth edition of the Brazilian Consensus for Autoantibodies Screening in HEp-2 Cells.Results and conclusion: the 4th ANA Consensus included three novel patterns into the existing algorithm (cytoplasmic Rods and Rings, nuclear Quasi-homogeneous, and CENP-F). Emphasis was given to the need of attention in describing the peculiar mixed pattern elicited by anti-DNA topoisomerase I (Scl-70) autoantibodies, comprising nuclear fine specked, nucleolar homogeneous pattern, NOR staining in metaphase plates, and cytoplasmic fine speckled patterns. the group also emphasized the need for continuous quality control in indirect immunofluorescence assays, the establishment of screening dilutions, as well as conjugate titration. An alert was made regarding the heterogeneity of commercial kits in defining patterns and the use of solid phase methodologies to determine the presence of autoantibodies. (C) 2014 Elsevier Editora Ltda. All rights reserved. PUC Goias, Goiania, Go, Brazil Fleury Med & Saude, São Paulo, Brazil Universidade Federal de São Paulo UNIFESP, Discipline Rheumatol, São Paulo, Brazil Univ Fed Uberlandia, Hosp Clin, Serv Rheumatol, BR-38400 Uberlandia, MG, Brazil Clin Doencas Reumat Porto Alegre, Ctr Diagnost Med & Rheuma, Porto Alegre, RS, Brazil Amaral Costa Med Diagnost, Belem, PA, Brazil Univ São Paulo, Fac Med, Hosp Clin, Lab Invest Med, São Paulo, Brazil Univ São Paulo, Fac Med, Hosp Clin, Lab Cent, São Paulo, Brazil Hosp Israelita Albert Einstein, Dept Clin Pathol, São Paulo, Brazil Inst Hermes Pardini, Belo Horizonte, MG, Brazil Univ São Paulo, Fac Med, São Paulo, Brazil Univ Fed Minas Gerais, Fac Med, Belo Horizonte, MG, Brazil Univ Catolica Brasilia, Brasilia, DF, Brazil Univ Fed Goias, Fac Med, Goiania, Go, Brazil Univ Sul Santa Catarina UNISUL, Florianopolis, SC, Brazil Univ Vale do Itajai UNIVALE, Florianopolis, SC, Brazil Univ Fed Espirito Santo, Vitoria, ES, Brazil EBMSP, Salvador, BA, Brazil Grp DASA, São Paulo, Brazil Escola Super Ciencias Saude Dist Fed, Brasilia, DF, Brazil Lab Sabin, Brasilia, DF, Brazil Universidade Federal de São Paulo UNIFESP, Discipline Rheumatol, São Paulo, Brazil Web of Science

ObjectiveThe Fourth Brazilian Consensus for Autoantibodies Screening in HEp-2 Cells (ANA) was held in Vitória, Espírito Santo, and aimed to discuss strategies and recommendations about the technique, standardization, interpretation and quality control of the indirect immunofluorescence reaction on HEp-2 cells.MethodsTwenty three ANA experts from university centers and private laboratories in different areas from Brazil discussed and agreed upon recommendations for the fourth edition of the Brazilian Consensus for Autoantibodies Screening in HEp-2 Cells.Results and conclusionThe 4th ANA Consensus included three novel patterns into the existing algorithm (cytoplasmic Rods and Rings, nuclear Quasi-homogeneous, and CENP-F). Emphasis was given to the need of attention in describing the peculiar mixed pattern elicited by anti-DNA topoisomerase I (Scl-70) autoantibodies, comprising nuclear fine specked, nucleolar homogeneous pattern, NOR staining in metaphase plates, and cytoplasmic fine speckled patterns. The group also emphasized the need for continuous quality control in indirect immunofluorescence assays, the establishment of screening dilutions, as well as conjugate titration. An alert was made regarding the heterogeneity of commercial kits in defining patterns and the use of solid phase methodologies to determine the presence of autoantibodies.

Objectives To investigate the association of comorbidities with mobility limitation and functional disability in patients with rheumatoid arthritis and to identify which comorbidity indicator is the most appropriate to determine this association. Methods Sixty rheumatoid arthritis patients were enrolled in a cross-sectional study for a period of 11 months. Comorbidities were assessed using three indicators: (i) the total number of comorbidities; (ii) the Charlson comorbidity index; and (iii) the functional comorbidity index. Disease activity was assessed using the Disease Activity Score 28. Functional capacity was measured using the Health Assessment Questionnaire, and mobility was measured using Timed Up and Go Test and Five-Times-Sit-to-Stand Test. Statistical analysis was performed using a stepwise log-linear multiple regression with a significance level of 5%. Results In the final model, only comorbidity was associated with mobility limitation. The functional comorbidity index score explained 19.1% of the variability of the Five-Times-Sit-to-Stand Test (coefficient of determination [ R 2 ] = 0.191) and 19.5% of the Timed Up and Go Test variability ( R 2 = 0.195). With regard to functional disability, the associated factors were comorbidity and disease activity, which together explained 32.9% of the variability of the Health Assessment Questionnaire score (adjusted R 2 = 0.329). Conclusion Comorbidities were associated with mobility limitation and functional disability in rheumatoid arthritis patients. The functional comorbidity index proved to be an appropriate comorbidity indicator to determine this association.

Resumo A síndrome antifosfolipíde (SAF) é uma doença autoimune sistêmica caracterizada por trombose arterial ou venosa recorrente e/ou morbidade gestacional e pela presença dos anticorpos antifosfolipídeos, podendo apresentar outras manifestações vasculares, como microangiopatia, arteriopatia crônica e SAF catastrófica. Determinados testes laboratoriais para a síndrome (por exemplo, o anticoagulante lúpico) podem sofrer interferência do uso de medicações anticoagulantes, dificultando o diagnóstico. A fisiopatologia da SAF é complexa, sendo enumerados no texto diversos mecanismos patogênicos relacionados à coagulação, ao endotélio e às plaquetas. Por fim, discutimos o tratamento da SAF de acordo com a presença e o tipo de manifestações clínicas, o uso dos anticoagulantes orais diretos e o manejo perioperatório de pacientes com SAF.