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Abstract Sjogren’s disease (SjD) is an autoimmune disease that is characterized not only by the sicca symptoms it causes but also by its systemic nature, which is capable of several and not yet fully understood extraglandular manifestations. To gain a clearer understanding of these manifestations as well as a better practical approach, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of epidemiologic and clinical features of the extraglandular manifestations present in ESSDAI (EULAR Sjogren´s syndrome disease activity index), followed by a voting panel with recommendations for clinical practice. This publication is complementary to others already published and covers cutaneous and hematological manifestations, with prevalence data generated by a meta-analysis of 13 clinical or laboratory manifestations and 6 clinical management recommendations. Sjogrens Sjogren s SjD (SjD nature approach metaanalysis meta analysis EULAR index, index , index) practice 1

https://doi.org/10.1186/s42358-024-00391-x

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/ meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic)
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Trevisani, Virginia Fernandes Moça

; Pinheiro, Aysa César

; Fialho, Sonia Cristina de Magalhães Souza

; Fernandes, Marilena Leal Mesquita Silvestre

; Pugliesi, Alisson

; Pasoto, Sandra Gofinet

; Lopes, Maria Lucia Lemos

; Guedes, Lissiane Karine Noronha

; Miyamoto, Samira Tatiyama

; Santos, Laura Caldas dos

; Appenzeller, Simone

; Fidelix, Tania

; Ribeiro, Sandra Lúcia Euzébio

; Brito, Danielle Christinne Soares Egypto de

; Libório-Kimura, Tatiana Nayara

; Santos, Maria Carmen Lopes Ferreira Silva

; Cantali, Diego Ustárroz

; Gennari, Juliana D.’Agostino

; Capobianco, Karina Gatz

; Civile, Vinicius Tassoni

; Pinto, Ana Carolina Pereira Nunes

; Rocha, Aline Pereira da

; Rocha Filho, César Ramos

; Oliveira, Fabiola Reis

; Valim, Valeria

Advances in Rheumatology

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2022, Volume 62 elocation 35

Abstract: En | Text: En | PDF: En

Abstract Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.

https://doi.org/10.1186/s42358-022-00267-y

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary sjogren syndrome: results of an epidemiologic systematic review/ meta-analysis and a consensus guideline from the Brazilian Society of Rheumatology (articular, pulmonary and renal)
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Trevisani, Virginia Fernandes Moça

; Pugliesi, Alisson

; Pasoto, Sandra Gofinet

; Lopes, Maria Lucia Lemos

; Guedes, Lissiane Karine Noronha

; Miyamoto, Samira Tatiyama

; Fernandes, Marilena Leal Mesquita Silvestre

; Fialho, Sonia Cristina de Magalhães Souza

; Pinheiro, Aysa César

; Santos, Laura Caldas dos

; Appenzeller, Simone

; Fidelix, Tania

; Ribeiro, Sandra Lúcia Euzébio

; Brito, Danielle Christinne Soares Egypto de

; Libório-Kimura, Tatiana Nayara

; Santos, Maria Carmen Lopes Ferreira Silva

; Cantali, Diego Ustárroz

; Gennari, Juliana D’Agostino

; Civile, Vinicius Tassoni

; Pinto, Ana Carolina Pereira Nunes

; Rocha-Filho, César Ramos

; Oliveira, Fabiola Reis

; Rocha, Aline Pereira da

; Valim, Valeria

Advances in Rheumatology

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2022, Volume 62 elocation 18

Abstract: En | Text: En | PDF: En

Abstract Sjogren’s Syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, these systemic manifestations are not routinely evaluated and there is no homogenous approach to their diagnosis or evaluation. To close this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of epidemiologic and clinical features of these manifestations and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. The first part of this guideline summarizes the most important topics, and 11 recommendations are provided for the articular, pulmonary, and renal care of SS patients.

https://doi.org/10.1186/s42358-022-00248-1

2021 recommendations of the Brazilian Society of Rheumatology for the gynecological and obstetric care of patients with Sjogren’s syndrome
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Oliveira, Fabiola Reis

; Valim, Valeria

; Pasoto, Sandra Gofinet

; Fernandes, Marilena Leal Mesquita Silvestre

; Lopes, Maria Lucia Lemos

; Souza Fialho, Sonia Cristina de Magalhães

; Pinheiro, Aysa César

; Santos, Laura Caldas dos

; Appenzeller, Simone

; Fidelix, Tania

; Ribeiro, Sandra Lucia Euzébio

; Brito, Danielle Christinne Soares Egypto de

; Libório, Tatiana

; Santos, Maria Carmen Lopes Ferreira Silva

; Tanure, Leandro

; Gennari, Juliana DAgostino

; Civile, Vinicius Tassoni

; Pinto, Ana Carolina Pereira Nunes

; Rocha Filho, César Ramos

; Miyamoto, Samira Tatiyama

; Guedes, Lissiane Karine Noronha

; Pugliesi, Alisson

; Trevisani, Virginia Fernandes Moça

Advances in Rheumatology

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2021, Volume 61 elocation 54

Abstract: En | Text: En | PDF: En

Abstract Sjogren’s syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs. Women with SS often experience gynecological symptoms due to the disease and need extra care regarding their sexual activity, reproductive health and during pregnancy, conditions that are not properly conducted in the clinical practice. To cover this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of symptoms, diagnosis, monitoring, prognosis, and treatment of these manifestations. A Focus Group meeting was held and included experts in the field and methodologists, based on a previously developed script, with themes related to the objective of the study. The most important topics were summarized and 11 recommendations were provided.

https://doi.org/10.1186/s42358-021-00208-1 54 downloads

Recommendations from the Brazilian society of rheumatology for the diagnosis of Sjögren's syndrome (Part I): glandular manifestations (systematic review)
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Trevisani, Virginia Fernandes Moça

; Pasoto, Sandra Gofinet

; Fernandes, Marilena Leal Mesquita Silvestre

; Lopes, Maria Lucia Lemos

; Fialho, Sonia Cristina de Magalhães Souza

; Pinheiro, Aysa César

; Santos, Laura Caldas dos

; Appenzeller, Simone

; Fidelix, Tania

; Ribeiro, Sandra Lucia Euzébio

; Brito, Danielle Christinne Soares Egypto de

; Libório, Tatiana

; Santos, Maria Carmen Lopes Ferreira Silva

; Tanure, Leandro

; Gennari, Juliana D Agostino

; Civile, Vinicius Tassoni

; Pinto, Ana Carolina Pereira Nunes

; Oliveira, Fabiola Reis

; Sousa, Jaqueline Martins de

; Miyamoto, Samira Tatiyama

; Valim, Valeria

Advances in Rheumatology

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2019, Volume 59 elocation 58

Abstract: En | Text: En | PDF: En

Abstract Background: Primary Sjögren's syndrome (pSS) is a systemic immune-mediated disease whose main characteristic is exocrine gland inflammation and, subsequent reduction in tear and saliva production. A delayed diagnosis is common due to the nonspecific clinical manifestations of disease. The aim of the present study was to develop recommendations for the diagnosis of glandular manifestations of pSS based on evidence and expert opinion. Main body of the abstract: We conducted a systematic literature review to retrieve the best evidence available on the accuracy of diagnostic tests for pSS. We also held two in-person meetings with experts (rheumatologists, pathologists, ophthalmologists and dentists) to establish their level of agreement using the Delphi method. Ultimately, we generated 18 recommendations that aim to facilitate the diagnosis of the glandular manifestations of pSS. Conclusion: The diagnosis of glandular manifestations of pSS is complex and multidisciplinary. It requires specific knowledge in the field of ophthalmology, immunology, pathology and imaging, making it compulsory for the rheumatologist to work with professionals from these different areas in order to improve accuracy and early diagnosis. Glandular dysfunction tests, ANA, RF, Anti-Ro, protein electrophoresis, urinalysis, blood count, C-Reactive protein, complement, testing for syphilis and viruses (HCV, HIV) and SGUS should be investigated when dryness or systemic manifestation are present. Minor salivary gland biopsy is recommended for all anti-Ro negative or incomplete criteria cases.

https://doi.org/10.1186/s42358-019-0102-8 878 downloads

Assessment of cardiovascular risk in patients with rheumatoid arthritis using the SCORE risk index
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Campos, Otávio Augusto Martins de

; Nazário, Nazaré Otília

; Fialho, Sônia Cristina de Magalhães Souza

; Fialho, Guilherme Loureiro

; Oliveira, Fernando José Savóia de

; Castro, Gláucio Ricardo Werner de

; Pereira, Ivânio Alves

Revista Brasileira de Reumatologia

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apr 2016, Volume 56 N. 2 Pages 138 - 144

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

RESUMO Introdução: Artrite reumatoide (AR) é uma doença autoimune que determina manifestações sistêmicas e está associada a aumento do risco de evento cardiovascular. Objetivo: Analisar o índice SCORE de predição de evento cardiovascular em pacientes do gênero feminino portadores de AR comparados com controles sem a doença. Métodos: Estudo de caso-controle com análise de 100 pacientes pareadas por gênero e idade versus 100 pacientes do grupo controle. Para a predição do risco de evento cardiovascular fatal em 10 anos, usamos os índices SCORE e SCORE modificado (mScore), conforme sugerido pela Eular, no subgrupo com 2 ou mais dos seguintes: duração da doença ≥ 10 anos, positividade para fator reumatoide e/ou anti-CCP e manifestações extra-articulares. Resultados: A prevalência das comorbidades analisadas foi similar nas pacientes com AR, em comparação com o grupo controle (p > 0,05). As médias do índice SCORE foram 1,99 (DP: 1,89) e 1,56 (DP: 1,87) nas portadoras de AR e nos controles (p = 0,06), respectivamente. Com o uso do índice mScore, nas pacientes com AR foi encontrada a média de 2,84 (DP: 2,86) versus 1,56 nos controles (DP: 1,87) (p = 0,001). Ao usar o índice SCORE, 11% dos portadores de AR foram classificados como de alto risco; com o índice mScores, 36% obtiveram essa classificação (p < 0,001). Conclusões: O índice SCORE é semelhante nos dois grupos, mas com a aplicação do índice mScore identificamos que os pacientes com AR têm maior risco de evento cardiovascular fatal em 10 anos, com ênfase na importância dos fatores inerentes à doença não mensurados no índice SCORE, mas considerados no índice mScore.

ABSTRACT Introduction: Rheumatoid arthritis is an autoimmune disease that causes systemic involvement and is associated with increased risk of cardiovascular disease. Objective: To analyze the prediction index of 10-year risk of a fatal cardiovascular disease event in female RA patients versus controls. Methods: Case-control study with analysis of 100 female patients matched for age and gender versus 100 patients in the control group. For the prediction of 10-year risk of a fatal cardiovascular disease event, the SCORE and modified SCORE (mSCORE) risk indexes were used, as suggested by EULAR, in the subgroup with two or more of the following: duration of disease ≥10 years, RF and/or anti-CCP positivity, and extra-articular manifestations. Results: The prevalence of analyzed comorbidities was similar in RA patients compared with the control group (p > 0.05). The means of the SCORE risk index in RA patients and in the control group were 1.99 (SD: 1.89) and 1.56 (SD: 1.87) (p = 0.06), respectively. The means of mSCORE index in RA patients and in the control group were 2.84 (SD = 2.86) and 1.56 (SD = 1.87) (p = 0.001), respectively. By using the SCORE risk index, 11% of RA patients were classified as of high risk, and with the use of mSCORE risk index, 36% were at high risk (p< 0.001). Conclusion: The SCORE risk index is similar in both groups, but with the application of the mSCORE index, we recognized that RA patients have a higher 10-year risk of a fatal cardiovascular disease event, and this reinforces the importance of factors inherent to the disease not measured in the SCORE risk index, but considered in mSCORE risk index.

https://doi.org/10.1016/j.rbre.2015.09.005 5117 downloads

Instrumentos, estratégias e método de abordagem qualitativa sobre tentativas e ideações suicidas de pessoas idosas
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Cavalcante, Fátima Gonçalves

; Minayo, Maria Cecília de Souza

; Gutierrez, Denise Machado Duran

; Sousa, Girliani Silva de

; Silva, Raimunda Magalhães da

; Moura, Rosylaine

; Meneghel, Stela Nazareth

; Grubits, Sonia

; Conte, Marta

; Cavalcante, Ana Célia Sousa

; Figueiredo, Ana Elisa Bastos

; Mangas, Raimunda Matilde do Nascimento

; Fachola, María Cristina Heuguerot

; Izquierdo, Giovane Mendieta

Ciência & Saúde Coletiva

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jun 2015, Volume 20 N. 6 Pages 1667 - 1680

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

O artigo analisa a qualidade e a consistência de um roteiro de entrevista compreensiva, adaptado para o estudo da tentativa de suicídio e sua ideação entre pessoas idosas e apresenta o método utilizado na aplicação desse instrumento. O objetivo é demonstrar como o uso da entrevista semiestruturada e sua forma de organização e análise de dados foram testados e aperfeiçoados por uma rede de pesquisadores de doze universidades ou centros de pesquisa do Brasil, Uruguai e Colômbia. O método envolveu a aplicação do instrumento em que se socializou um manual de instruções sobre a coleta, sistematização e análise de dados. A metodologia foi aplicada em 67 entrevistas com idosos na faixa de 60 anos ou mais e em 34 entrevistas com profissionais da saúde em treze municípios brasileiros, em Montevidéu e Bogotá, permitindo a verificação da consistência do instrumento usado e a aplicabilidade do método, durante o processo e ao final. O roteiro aperfeiçoado e as instruções para replicá-lo e analisá-lo são aqui apresentados. Os resultados apontam a adequação e a credibilidade dessa abordagem metodológica testada e qualificada de modo interdisciplinar e interinstitucional.

The article analyses the quality and consistency of a comprehensive interview guide, adapted to study attempted suicide and its ideation among the elderly, and imparts the method followed in applying this tool. The objective is to show how the use of a semi-structured interview and the organization and data analysis set-up were tested and perfected by a network of researchers from twelve universities or research centers in Brazil, Uruguay and Colombia. The method involved application and evaluation of the tool and joint production of an instruction manual on data collection, systematization and analysis. The methodology was followed in 67 interviews with elderly people of 60 or older and in 34 interviews with health professionals in thirteen Brazilian municipalities and in Montevideo and Bogotá, allowing the consistency of the tool and the applicability of the method to be checked, during the process and at the end. The enhanced guide and the instructions for reproducing it are presented herein. The results indicate the suitability and credibility of this methodological approach, tested and certified in interdisciplinary and interinstitutional terms.

https://doi.org/10.1590/1413-81232015206.03022015 10417 downloads

Instrumentos, estratégias e método de abordagem qualitativa sobre tentativas e ideações suicidas de pessoas idosas
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Cavalcante, Fátima Gonçalves

; Minayo, Maria Cecília de Souza

; Gutierrez, Denise Machado Duran

; Sousa, Girliani Silva de

; Silva, Raimunda Magalhães da

; Moura, Rosylaine

; Meneghel, Stela Nazareth

; Grubits, Sonia

; Conte, Marta

; Cavalcante, Ana Célia Sousa

; Figueiredo, Ana Elisa Bastos

; Mangas, Raimunda Matilde do Nascimento

; Fachola, María Cristina Heuguerot

; Izquierdo, Giovane Mendieta

Ciência & Saúde Coletiva

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jun 2015, Volume 20 N. 6 Pages 1667 - 1680

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

https://doi.org/10.1590/1413-81232015206.03022015 1819 downloads

Aterosclerose subclínica em pacientes com espondilite anquilosante: há um papel para a inflamação?
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Valente, Renato Leandro Mattar

; Valente, Jamil Mattar

; Castro, Gláucio Ricardo Werner de

; Zimmermann, Adriana Fontes

; Fialho, Sonia Cristina de Magalhães Souza

; Pereira, Ivânio Alves

Revista Brasileira de Reumatologia

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oct 2013, Volume 53 N. 5 Pages 377 - 381

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVOS: Avaliar a prevalência de aterosclerose subclínica em pacientes com espondilite anquilosante (EA) em comparação com controles com fatores de risco cardiovasculares similares. MÉTODOS: Foram recrutados 42 pacientes consecutivos com EA e 42 controles equiparados para idade (43,3 ± 11,7 vs. 43,7 ± 11,3, P = 0,89), gênero, tabagismo, diabetes mellitus e hipertensão arterial. Qualquer participante seria excluído se estivesse presente uma história pessoal de doença cardiovascular (CV). Foi preenchido um questionário registrando dados demográficos e histórias médica e de medicação. Foram determinados: pressão arterial, circunferência abdominal, altura e peso. O perfil lipídico foi determinado em uma amostra de sangue com 12 horas em jejum. Foi realizada uma análise ultrassonográfica da artéria carótida comum por um observador desconhecedor da pesquisa. Foi medida a distância entre a interface lúmen-íntima e a borda de ataque da interface média-adventícia (EIM) e os participantes também foram avaliados para presença de placas. RESULTADOS: A análise comparativa dos fatores de risco demográficos e cardiovasculares entre pacientes com EA e controles não revelou diferenças estatisticamente significativas. Também não foram observadas diferenças significativas entre grupos para TC, HDL-C, T-C/ HDL-C, LDL-C, triglicerídeos ou frequência de dislipidemia. As medidas de EIM não foram diferentes em EA e controles (0,62 ± 0,09 vs. 0,61 ± 0,09, P = 0,39) e nem as frequências de placas (19% vs. 17%, P = 0,78). CONCLUSÕES: A aterosclerose subclínica avaliada por meio de imagens ultrassonográficas da carótida não foi mais prevalente no grupo EA, em comparação com os controles com riscos cardiovasculares similares. Nossas observações podem implicar que os fatores de risco CV podem ter mais influência no sistema CV versus a própria EA. Esses achados devem ser confirmados em uma população maior, por meio de um estudo prospectivo.

OBJECTIVES: To evaluate the prevalence of subclinical atherosclerosis in patients with ankylosing spondylitis (AS) in comparison to controls with similar cardiovascular risk factors. METHODS: Forty-two consecutive patients with AS and 42 controls matched for age (43.3 ± 11.7 vs. 43.7 ± 11.3, P = 0.89), gender, smoking, diabetes mellitus and arterial hypertension were enrolled. Participants were excluded if a personal cardiovascular disease (CV) history was present. A questionnaire recording demographic data, medical and medication history was fulfilled. Blood pressure, abdominal circumference, height and weight were measured. Lipid profile was determined in a 12-hour fastened blood sample. Ultrasound analysis of the common carotid artery was performed by one blind observer. The distance between the lumen-intima interface and the leading edge of the media-adventitia interface (IMT) was measured and participants were also evaluated for the presence of plaques. RESULTS: The comparative analysis of demographic and cardiovascular risk factors between AS patients and controls did not reveal statistically significant differences. Also, no significant differences between groups were observed for TC, HDL-C, T-C/HDL-C, LDL-C, triglycerides, or dyslipidemia frequency. IMT measures were not different in AS and controls (0.62 ± 0.09 vs. 0.61 ± 0.09, P = 0.39) as well as plaques frequencies (19% vs. 17%, P = 0.78). CONCLUSIONS: Subclinical atherosclerosis assessed through carotid ultrasound imaging was not more prevalent in the AS group when compared to controls with similar cardiovascular risks. Our observations may imply that CV risk factors may have more influence on the CV system than AS itself. These findings should be confirmed in a larger population with a prospective study design.

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10.

Lesões destrutivas da linha média induzidas por cocaína com ANCA positivo mimetizando a granulomatose de Wegener
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Stahelin, Letícia

; Fialho, Sonia Cristina de Magalhães Souza

; Neves, Fabrício Souza

; Junckes, Larissa

; Werner de Castro, Gláucio Ricardo

; Pereira, Ivânio Alves

Revista Brasileira de Reumatologia

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jun 2012, Volume 52 N. 3 Pages 434 - 437

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

O uso crônico de cocaína por inalação pode causar lesões destrutivas de linha média (LDLMIC), que podem ser difíceis de distinguir das lesões da granulomatose de Wegener (GW) nos ouvidos, nariz e garganta. Descrevemos o caso de uma paciente de 43 anos admitida com história de dois anos de obstrução nasal e rinorreia. Ela havia recebido o diagnóstico de GW há cinco meses e estava em tratamento com prednisona e ciclofosfamida. Ao exame físico apresentava perfuração de septo nasal e palato. Exames de laboratório mostraram elevação das proteínas de fase aguda e teste p-ANCA positivo. Ensaios ELISA antiproteinase 3 e mieloperoxidase foram negativos. Tomografia computadorizada (TC) dos seios paranasais mostrou destruição de septo nasal e palato, bem como sinusite maxilar bilateral. TC de tórax resultou normal. Biópsia da mucosa nasal revelou infiltrado inflamatório sem granuloma ou vasculite. Quando questionada, admitiu ser usuária de cocaína há cinco anos. Os imunossupressores foram suspensos e a paciente não mais fez uso da droga. Ela está sendo monitorada há seis meses e não desenvolveu novas lesões ou sintomas de outros órgãos. O diagnóstico diferencial em pacientes com LDLMIC pode ser desafiador. A avaliação deve incluir pesquisa de uso intranasal de cocaína. Embora o teste de ANCA não diferencie claramente o ANCA encontrado em alguns pacientes com LDLMIC daqueles em pacientes com GW, o envolvimento localizado e os achados de biópsia não típicos de vasculite granulomatosa de pequenos vasos devem ser reconhecidos como características das lesões induzidas por cocaína.

Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL), which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegener's Granulomatosis (WG). We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been diagnosed with WG for five months, being on prednisone and cyclophosphamide. On her physical examination, perforation of her nasal septum and palate was observed. Laboratory tests showed elevated acute phase proteins and a positive p-ANCA test. ELISA assays anti-proteinase 3 and myeloperoxidase were negative. The paranasal sinus computed tomography (CT) showed destruction of the nasal septum and palate, in addition to bilateral maxillary sinusitis. Chest CT was normal. Nasal mucosal biopsy revealed an inflammatory infiltrate, with neither granuloma nor vasculitis. When questioned, she admitted being a cocaine user for five years. Medical therapy and cocaine use were withdrawn. She has been followed up for six months and no other lesion or other organ symptoms occurred. Differential diagnosis in patients with midline destructive lesions can be very challenging. Evaluation should include enquiry about intranasal use of cocaine. Although ANCA testing does not clearly differentiate the ANCA found in some patients with CIMDL from those found in WG patients, the localized involvement and the biopsy findings non-characteristic of small vessel granulomatous vasculitis should be recognized as features for cocaine-induced lesions.

40412 downloads

11.

Micofenolato mofetil na síndrome de Sjögren primária: uma opção para o tratamento da agranulocitose
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Fialho, Sonia Cristina de Magalhães Souza

; Bergamaschi, Samuel

; Neves, Fabrício Souza

; Zimmermann, Adriana Fontes

; Castro, Gláucio Ricardo Werner de

; Pereira, Ivânio Alves

Revista Brasileira de Reumatologia

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apr 2012, Volume 52 N. 2 Pages 297 - 299

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

A síndrome de Sjögren (SS) é uma doença autoimune caracterizada pela presença de infiltrado linfocítico nas glândulas salivares e lacrimais. Manifestações hematológicas da síndrome de Sjögren primária (SSp) geralmente consistem em anemia leve, trombocitopenia, neutropenia moderada e linfopenia. Agranulocitose é raramente descrita e, em geral, responde bem ao tratamento de imunossupressão. Neste trabalho, descrevemos o caso de uma paciente portadora de SSp que apresentou quadro de agranulocitose refratária ao tratamento. A biópsia de medula revelou medula óssea hipocelular com maturação normal da série granulocítica. A paciente foi sucessivamente tratada com prednisona em altas doses, fator estimulador de colônia de macrófagos e ciclosporina, todos sem resposta hematológica. Micofenolato mofetil (MMF) foi iniciado, e após dois meses houve aumento na contagem das células brancas. Após um ano de seguimento a paciente não apresentou novos episódios de neutropenia, nem complicações infecciosas. Concluímos que, na agranulocitose refratária associada à SSp, o tratamento com MMF pode ser uma opção eficaz e bem tolerada.

The Sjögren's syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of salivary and lacrimal glands. Hematological manifestations of primary SS (pSS) usually consist of mild anemia, thrombocytopenia, moderate neutropenia, and lymphopenia. Agranulocytosis is rarely reported and usually responds to immunosuppression. We report the case of a pSS patient who presented with refractory agranulocytosis. Bone marrow biopsy disclosed a hypocellular bone marrow with normal maturation of the granulocytic series. The patient was successively treated with high-dose prednisone, granulocyte-macrophage colony stimulation factor, and cyclosporine, with no hematological response. Mycophenolate mofetil (MMF) was initiated and after two months there was a rise on the white blood cell count. After one year of follow-up, she had neither further neutropenia episodes, nor infectious complications. We conclude that, in pSS refractory agranulocytosis, MMF can be an effective and well-tolerated treatment option.

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12.

Avaliação do sistema musculoesquelético na unidade de emergência
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Fialho, Sonia Cristina de Magalhães Souza

; Castro, Gláucio Ricardo Werner de

; Zimmermann, Adriana Fontes

; Ribeiro, Giovana Gomes

; Neves, Fabrício Souza

; Pereira, Ivânio Alves

; Fialho, Guilherme Loureiro

Revista Brasileira de Reumatologia

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jun 2011, Volume 51 N. 3 Pages 244 - 248

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVO: As condições musculoesqueléticas possuem um enorme e crescente impacto no mundo. A despeito disso, alguns médicos não estão confiantes em suas próprias habilidades para a realização do exame clínico musculoesquelético. Este estudo teve como objetivo avaliar a prevalência dos sintomas musculoesqueléticos em uma unidade de emergência e a frequência de descrição do exame físico musculoesquelético nesses casos. MÉTODOS: Trata-se de estudo transversal. Foi realizada uma análise sistemática das fichas de atendimento na unidade de emergência do hospital da Universidade Federal de Santa Catarina, Brasil, de 24 a 30 de abril de 2009. RESULTADOS:Foram analisadas 392 fichas de atendimento, onde 41,5% dos pacientes eram do sexo masculino e a idade média de 38,7 ± 17,2 anos. Sessenta e nove dos 392 pacientes (17,6%) apresentaram-se com queixa musculoesquelética. A queixa musculoesquelética mais comum foi lombalgia (33/69). Somente 49,2% dos pacientes que apresentavam distúrbios musculoesqueléticos como queixa principal tiveram exame físico específico descrito nas fichas. Pacientes com queixas musculoesqueléticas tiveram menor frequência de registro de exame abdominal (46% versus 62%, P = 0,01) e sinais vitais (46% versus 66%, P = 0,00), porém maior frequência de registro do exame musculoesquelético (49% versus 0,6%, P = 0,00). CONCLUSÕES:Este estudo confirma outras observações em todo o mundo. Queixas musculoesqueléticas são frequentes em uma unidade de emergência e, apesar disso, sugere-se que os sintomas musculoesqueléticos são insuficientemente avaliados, o que pode estar relacionado a uma educação médica insuficiente. É fundamental que escolas médicas coloquem maior ênfase nessas condições para que jovens médicos estejam mais preparados para lidar com essas doenças comuns.

OBJECTIVE: Musculoskeletal conditions have an enormous and growing impact worldwide. In spite of that, some clinicians are not confident in their own musculoskeletal examination skills. This study aimed to evaluate the prevalence of musculoskeletal symptoms in an emergency room, and the frequency of musculoskeletal physical examination description on those cases. METHODS: This was a cross-sectional study. We performed a systematic analysis of medical files at the emergency room of the University Hospital of the Federal University of Santa Catarina, Brazil, from April 24th to 30th, 2009. RESULTS: We analyzed 392 files, where 41.5% of patients were male and mean age was 38.7 ± 17.2 years-old. Sixty nine out of 392 patients (17.6%) presented with a musculoskeletal complaint. The most common musculoskeletal complaint was low back pain (33/69). Only 49.2% of patients with a musculoskeletal chief complaint had a specific physical examination registered on the files. Patients with musculoskeletal complaints had lower registrations of abdominal examination (46% versus 62%, P = 0.01) and vital signs (46% versus 66%, P = 0.002), but a higher frequency of musculoskeletal examination registration (49% versus 0.6%, P = 0.00). CONCLUSIONS: Our study confirms other observations worldwide. Musculoskeletal complaints are frequent in a emergency room setting and in spite of that it is suggested that musculoskeletal symptoms are poorly evaluated, which is probably related to an insufficient musculoskeletal education. It is essential that medical schools place more emphasis on these conditions so that young physicians will be more prepared to deal with these common diseases.

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13.

Toxicidade hepática é rara em pacientes com artrite reumatoide usando terapia combinada de leflunomida e metotrexato
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Alves, Jorge Augusto Nunes Rodrigues

; Fialho, Sonia Cristina de Magalhães Souza

; Morato, Edelton Flávio

; Castro, Gláucio RicardoWerner de

; Zimmermann, Adriana Fontes

; Ribeiro, Giovana Gomes

; Neves, Fabrício Souza

; Pereira, Ivânio Alves

Revista Brasileira de Reumatologia

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apr 2011, Volume 51 N. 2 Pages 141 - 144

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVO: De acordo com alguns estudos, a associação de leflunomida (LEF) a pacientes portadores de artrite reumatoide não responsivos a metotrexato (MTX) aumentou a eficácia do tratamento, elevando, porém, o risco de toxicidade hepática. Este estudo objetiva avaliar a incidência de toxicidade hepática no tratamento da artrite reumatoide ativa usando terapia combinada de LEF e MTX em comparação com monoterapia com MTX. MÉTODOS: Entre fevereiro e setembro de 2009, foram arrolados 97 pacientes consecutivos acompanhados pelo Hospital Universitário da Universidade Federal de Santa Catarina, Brasil. Pacientes com artrite reumatoide em uso de MTX somente ou em combinação com LEF tiveram seus prontuários sistematicamente revisados. As enzimas alanino/aspartato aminotransferases foram analisadas retrospectivamente desde o tratamento com MTX ou MTX mais LEF. Hepatotoxicidade foi definida como um aumento das enzimas hepáticas acima de duas vezes o limite superior da normalidade. RESULTADOS: 71 pacientes foram incluídos no estudo: 36,6% usavam 20-25 mg/semana de MTX e 63,4% usavam 20-25 mg/semana de MTX associado a 20 mg/ dia de LEF. Dos pacientes em terapia combinada, 11,1% tinham níveis anormais das enzimas hepáticas versus 11,5% daqueles em monoterapia (P = 1,0). Níveis anormais de aminotransferases têm sido observados em pacientes com artrite reumatoide tanto em monoterapia com MTX quanto com LEF. Em nosso estudo, não encontramos diferença entre as percentagens de elevação das aminotransferases em pacientes tratados somente com MTX ou com terapia combinada. CONCLUSÃO: A combinação de MTX e LEF em pacientes com artrite reumatoide é geralmente segura e bem tolerada.

OBJECTIVE: Some studies have reported that adding leflunomide (LEF) to the treatment of rheumatoid arthritis (RA) in patients who do not respond to methotrexate (MTX) improved efficacy but increased the risk of liver toxicity. This study aimed at assessing the incidence of liver toxicity in patients with active RA using the LEF and MTX combination therapy in comparison with that of patients on MTX monotherapy. METHODS: Between February and September 2009, 97 consecutive patients followed up at the University Hospital of the Universidade Federal de Santa Catarina, Brazil, were enrolled. RA patients on MTX alone or using the LEF and MTX combination had their medical records systematically reviewed. The alanine/aspartate aminotransferase enzymes were retrospectively analyzed since the beginning of treatment with MTX or MTX plus LEF. Hepatotoxicity was defined as an increase of at least two-fold the upper limits of normal of the liver enzymes. RESULTS: 71 RA patients were included in the study: 36.6% were using 20-25 mg/week of MTX alone and 63.4% were using 20-25 mg/week of MTX plus 20 mg/day of LEF. Of the patients on the combination therapy, 11.1% had abnormal levels of liver enzymes versus 11.5% of the patients on monotherapy (P = 1.0). Abnormal aminotransferase levels have been seen with both MTX and LEF monotherapies in patients with RA. In our study, no difference was found between the percentages of aminotransferase elevations of patients being treated with MTX alone or in combination with LEF. CONCLUSION: The combination of MTX and LEF in RA patients is generally safe and well tolerated.

34736 downloads

14.

Síndrome de Sjögren primária com vasculite cutânea manifestada por úlceras em membro inferior
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Souza, Sonia Cristina de Magalhães

; Kuruma, Katia Akemi Miyazato

; Andrade, Danieli Castro Oliveira de

; Azevedo, Pedro Ming

; Figueiredo, Camille Pinto

; Borba, Eduardo Ferreira

; Gonçalves, Célio Rodrigues

; Borges, Cláudia Teresa Lobato

Revista Brasileira de Reumatologia

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apr 2004, Volume 44 N. 2 Pages 175 - 178

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

A síndrome de Sjögren (SS) primária é uma doença autoimune cujo espectro de manisfestação clínica estende-se desde um acometimento órgão-específico (exocrinopatia auto-imune) até um processo sistêmico. O envolvimento da pele é bastante comum, e a freqüência de doença vascular inflamatória é estimada entre 20% e 30%. Duas formas clínicas específicas de vasculite cutânea, a púrpura palpável e urticária crônica são predominantes, mas eritema multiforme, eritema perstans, eritema nodoso, mácula eritematosa e nódulo subcutâneo também já foram descritos. Os autores descrevem o caso de uma paciente de 46 anos, que desenvolveu quadro de síndrome de Sjögren primária, com manifestações oculares, orais, articulares e alterações laboratoriais (FAN, anti-Ro, fator reumatóide positivos, e hipergamaglobulinemia). Após dez anos do diagnóstico, apresentou úlceras em membro inferior, cuja biópsia confirmou tratar-se de lesão tipo vasculítica, com excelente resposta ao tratamento com ciclofosmida endovenosa. Foram descritos na literatura apenas dois relatos de caso de pacientes com úlceras em membros inferiores, como acometimento cutâneo da SS. Os autores ressaltam a importância do diferencial de úlceras em membros inferiores como acometimento cutâneo da SS primária.

Primary Sjögren's Syndrome (pSS) is an autoimmune disease with a large spectrum of clinical manifestations extending from an organ-specific involvement to a systemic process. The skin is affected quite commonly and the estimated frequency of inflammatory vascular lesions is from 20% to 30%. Two specific, clinically recognizable forms of cutaneous vasculitis predominate, palpable purpura and chronic urticaria, but erythema multiforme, erythema perstans, erythema nodosum, erithematous macules and subcutaneous nodules have also been described. The authors report the case of a 46-year-old female patient, diagnosed as primary SS, who presented ocular and oral symptoms, poliarthritis and laboratory alterations (with a positive ANA, anti-SSA, rheumatoid factor, and hypergammaglobulinemia). Ten years after the diagnosis, she presented leg ulcers. The biopsy confirmed the presence of vasculitic process, and the ulcers improved rapidly after the treatment with endovenous cyclophosphamide. There are only two reports of chronic ulceration of the legs as cutaneous manifestation of SS. The authors stress the importance of considering ulcers in the differential diagnosis of cutaneous involvement of primary SS.

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15.

The effect of intravenous zoledronic acid on glucocorticoid-induced multiple vertebral fractures in juvenile systemic lupus erythematosus
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Souza, Sonia Cristina de Magalhães

; Borges, Claudia Tereza Lobato

; Jorgetti, Vanda

; Pereira, Rosa Maria Rodrigues

Revista do Hospital das Clínicas

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2004, Volume 59 N. 5 Pages 302 - 305

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

Glicocorticóides são fármacos comumente usados no tratamento de pacientes lúpicos, porém apresentam efeitos adversos importantes, principalmente a osteoporose e fraturas. O tratamento da osteoporose em pacientes jovens deve ser eficaz e não prejudicial ao crescimento e remodelamento ósseo. Os bisfosfonatos são drogas que reduzem a incidência de fraturas, mas seu uso em crianças e adolescentes ainda é controverso, devido a seus possíveis efeitos adversos no esqueleto em crescimento. Estudos recentemente publicados demonstraram que o crescimento linear se manteve normal com o uso de bisfosfonatos, não havendo supressão excessiva do remodelamento ósseo ou defeitos de mineralização. O ácido zoledrônico é um novo bisfosfonato endovenoso aprovado pelo FDA para o uso na hipercalcemia das neoplasias e parece ser um tratamento eficaz para a osteoporose pós-menopáusica. Os autores descrevem um caso de uma adolescente lúpica que desenvolveu múltiplas fraturas vertebrais induzidas pelo glicocorticóide e obteve importante melhora clínica e densitométrica após o tratamento com o ácido zoledrônico.

Glucocorticoids are widely used in the treatment of lupus patients, and adverse effects, which include osteoporosis and associated fractures, are frequent. Treatment of osteoporosis of young patients should be effective and not harmful to bone growth and remodeling. Bisphosphonates are drugs that decrease the incidence of bone fractures, but their use in juvenile patients is still controversial because of their possible side effects on the growing skeleton. However, recently published studies showed that linear growth continued normally after treatment with these drugs, and there was no excessive suppression of bone remodeling or mineralization defects. Zoledronic acid is a new intravenous bisphosphonate that has been approved by the US FDA for use with hypercalcemia of malignancies and might be an effective treatment for postmenopausal osteoporosis. The authors report a case of a young girl with systemic lupus who developed multiple vertebral collapses due to glucocorticoid therapy, and zoledronic acid was used producing significant clinical and densitometric improvement.

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