Recommendations on cutaneous and hematological manifestations of Sjögren's disease by the Brazilian Society of Rheumatology.

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.

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).

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.

Perception of Portuguese Consumers Regarding Food Labeling.

Food labels are the first information tool used by consumers in the purchase and consumption of food products. Food labeling is a tool that can influence the consumers' perception of quality and, in turn, their food choice. This study characterizes and demonstrates the importance of labeling and the degree of consumers' perception and literacy about food labels through the application of an online questionnaire. The results obtained, in a sample of n = 467, showed that Portuguese consumers have the habit of reading the label and recognizing its importance but do not understand all the information contained in the label. They have an easier time understanding the front-of-pack labeling systems, especially those presented through symbols/colors. Thus, it demonstrates the need for greater education and literacy in the areas of food and nutrition so that through the reading and interpretation of labels, consumers can make informed food choices.

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).

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.

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)

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.

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)

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.

2021 recommendations of the Brazilian Society of Rheumatology for the gynecological and obstetric care of patients with Sjogren's syndrome.

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.

2021 recommendations of the Brazilian Society of Rheumatology for the gynecological and obstetric care of patients with Sjogren's syndrome

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.

Recommendations from the Brazilian society of rheumatology for the diagnosis of Sjögren's syndrome (Part I): glandular manifestations (systematic review).

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. 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.

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

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.

Assessment of cardiovascular risk in patients with rheumatoid arthritis using the SCORE risk index.

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.

Assessment of cardiovascular risk in patients with rheumatoid arthritis using the SCORE risk index / Avaliação do risco cardiovascular de pacientes com artrite reumatoide utilizando o índice SCORE

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.

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.

Pneumonia por Legionella após uso de Infliximabe em paciente com Artrite Reumatoide / Legionella pneumonia after infliximab in a patient with Rheumatoid Arthritis

Os antagonistas do fator de necrose tumoral (anti-TNF) têm sido utilizados com sucesso em várias doenças inflamatórias crônicas, como artrite reumatoide (AR), mas alguns estudos observaram a ocorrência de infecções por patógenos intracelulares em pacientes medicados com anti-TNF. Relatamos um caso de paciente mulher com diagnóstico prévio de AR durante 16 anos e que estava sendo medicada com várias drogas antirreumáticas modificadoras de doença (DARMDs), tendo como resultado o insucesso terapêutico, sendo em seguida tratada com infliximab. Depois de transcorridos 15 dias da segunda dose, a paciente foi acome- tida por dor torácica ventilatório-dependente, tosse seca e dispneia. Foi hospitalizada, e o diagnóstico de pneumonia por Legionella pneumophila foi confirmado pela presença do antí- geno de Legionella na urina. TNF é uma citocina inflamatória que também promove inibição do crescimento bacteriano de patógenos intracelulares, e sua inibição parece aumentar a sensibilidade a essas infecções em alguns pacientes.

The antagonists of tumour necrosis factor (anti-TNF) have been successfully used in several chronic inflammatory diseases such as Rheumatoid Arthritis (RA), but some studies have observed the development of infections by intracellular pathogens in patients using anti-TNF. We report a case of a female patient with previous diagnosis of RA for 16 years that used several disease-modifying anti-rheumatic drugs (DMARDs) that resulted in treatment failure, and then was treated with infliximab. After fifteen days of the second dose, the patient developed ventilatory-dependent chest pain, dry cough and dyspnea. She was hospitalized, and the diagnosis of pneumonia by Legionella pneumophila was confirmed by the presence of Legionella antigen in an urine test. TNF is an inflammatory cytokine that also acts inhibiting the bacterial growth of intracellular pathogens, and its inhibition seems to increase susceptibility to these infections in some patients.

[Legionella pneumonia after infliximab in a patient with rheumatoid arthritis]. / Pneumonia por Legionella após uso de Infliximabe em paciente com Artrite Reumatoide.

The antagonists of tumour necrosis factor (anti-TNF) have been successfully used in several chronic inflammatory diseases such as Rheumatoid Arthritis (RA), but some studies have observed the development of infections by intracellular pathogens in patients using anti-TNF. We report a case of a female patient with previous diagnosis of RA for 16 years that used several disease-modifying anti-rheumatic drugs (DMARDs) that resulted in treatment failure, and then was treated with infliximab. After fifteen days of the second dose, the patient developed ventilatory-dependent chest pain, dry cough and dyspnea. She was hospitalized, and the diagnosis of pneumonia by Legionella pneumophila was confirmed by the presence of Legionella antigen in an urine test. TNF is an inflammatory cytokine that also acts inhibiting the bacterial growth of intracellular pathogens, and its inhibition seems to increase susceptibility to these infections in some patients.

Subclinical atherosclerosis in ankylosing spondylitis: is there a role for inflammation?

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.

Aterosclerose subclínica em pacientes com espondilite anquilosante: há um papel para a inflamação? / Subclinical atherosclerosis in ankylosing spondylitis: is there a role for inflammation?

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.

Lesões destrutivas da linha média induzidas por cocaína com ANCA positivo mimetizando a granulomatose de Wegener / Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis

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.

Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis.

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.

Tumor markers are elevated in patients with rheumatoid arthritis and do not indicate presence of cancer.

AIM: To investigate serum levels of tumor markers in patients with rheumatoid arthritis (RA) and their association with disease activity or the presence of cancer. METHOD: We performed an observational cohort study including 100 patients with RA and control subjects. Serum levels of tumor markers carcinoembryonic antigen (CEA), cancer antigen (CA) 125, CA 19-9 and CA 15-3 were evaluated along with clinical and laboratorial RA data. Association tests between tumor markers levels and RA disease activity parameters were performed. Patients with abnormal tests were submitted to further investigation, including chest X-ray, colonoscopy, abdominal ultrasonography, upper gastrointestinal endoscopy and mammography, depending on the type of tumor marker that was elevated. RESULTS: Patients with RA had high levels of CEA and CA 19-9 more frequently than controls (P < 0.05). No correlation was found between tumor markers and RA disease activity assessed by the Disease Activity Score 28. Two neoplasms were found, but only one was related to high tumor marker (an ovarian carcinoma with high CA 125 levels). CONCLUSION: High tumor markers were frequently found in RA patients, even with controlled disease and were not related to actual cancer. Therefore, small increases of these markers in RA cases probably do not warrant a search for an occult neoplasm.