Reliability assessment of the OMERACT whole-body magnetic resonance imaging scoring system for juvenile idiopathic arthritis.

Inter-reader reliability of a new scoring system for evaluating joint inflammation and enthesitis in whole body MRI (WBMRI) in juvenile idiopathic arthritis was tested. The scoring system grades 732 item-region combinations of bone marrow and soft tissue changes for commonly involved joints and entheseal sites. Five radiologists rated 17 WBMRI scans through an online rating platform. Item-wise reliability was calculated for 117 items with non-zero scores in >10 % of readings. Interquartile ranges of the five-reader Kappa reliability coefficients were 0.58-0.73 (range: 0.36-0.88) for the joints, 0.65-0.81 (range: 0.39-0.95) for the entheses, and 0.62-0.75 (range: 0.60-0.76) for chronic nonbacterial osteomyelitis-like lesions.

Assessing the Utility of the Montreal Cognitive Assessment in Screening for Cognitive Impairment in Patients With Systemic Lupus Erythematosus.

OBJECTIVE: Screening for cognitive impairment (CI) in systemic lupus erythematosus (SLE) relies on the American College of Rheumatology (ACR) neuropsychological battery (NB). By studying the concurrent criterion validity, our goal was to assess the Montreal Cognitive Assessment (MoCA) as a screening tool for CI compared to the ACR-NB and to evaluate the added value of the MoCA to the Automated Neuropsychological Assessment Metrics (ANAM). METHODS: A total of 285 adult SLE patients were administered the ACR-NB, MoCA, and ANAM. For the ACR-NB, patients were classified as having CI if there was a Z score of ≤-1.5 in ≥2 domains. The area under the curve (AUC) and sensitivities/specificities were determined. A discriminant function analysis was applied to assess the ability of the MoCA to differentiate between CI, undetermined CI, and non-CI patients. RESULTS: CI was not accurately identified by the MoCA compared to the ACR-NB (AUC of 0.66). Sensitivity and specificity were poor at 50% and 69%, respectively, for the cutoff of 26, and 80% and 45%, respectively, for the cutoff of 28. The MoCA had a low ability to identify CI status. The addition of the MoCA to the ANAM led to improvement on the AUC by only 2.5%. CONCLUSION: The MoCA does not have adequate concurrent criterion validity to accurately identify CI in patients with SLE. The low specificity of the MoCA may lead to overdiagnosis and concern among patients. Adding the MoCA to the ANAM does not substantially improve the accuracy of the ANAM. These results do not support using the MoCA as a screening tool for CI in patients with SLE.

Cross-cultural adaptation and initial validation of the Brazilian-Portuguese version of the pediatric automated neuropsychological assessment metrics.

Automated neuropsychiatric batteries have been used in research and clinical practice, including for chronic diseases, such as Systemic Lupus Erythematosus. The Pediatric Automated Neuropsychological Assessment Metrics battery (Ped-ANAM), originally developed for use in American-English speaking individuals, allows tracking of cognitive functions. It can be applied to people over 9 years old. The aim of this study was to translate and present initial validation data from the Ped-ANAM into Brazilian-Portuguese. We translated the battery according to Beaton's guidelines. Psychometric properties were tested, internal consistency was analyzed by Cronbach's alpha coefficient, test-retest reliability by the intraclass correlation coefficient (ICC). Further, we measured the test execution speed at both times as a temporal stability. Principal component analysis (PCA) was used for structural validity. Evidence of construct validity was assessed through assessment of the relationships with the Wechsler Intelligence Scales. All participants prior to the start of study related activities signed an informed consent form approved by the local ethics committee. A sample of 230 individuals [mean (range) of age: 23 (9 to 60) years; 65% females] was included; a subset of 51 individuals [mean (range) of age: 18 (9 to 57) years, 59% female] completed the Ped-ANAM twice to assess test-retest reliability, and another subset of 54 individuals [mean (range) of age: 20.4 (7 to 62) years; 67% female] completed the Wechsler Intelligence Scales for Children and Adult for assessment of the Ped-ANAM's construct validity. Our results suggest that the internal consistency of the Ped-ANAM (Cronbach's α = 0.890) and its subtest test-retest reliability were excellent (ICC: 0.59 to 0.94). There was no clustering in the Principal Components Analysis, suggestive of non-grouping of the evaluated variables. Construct validity assessment to the Wechsler Scales showed expected ranges of low to strong correlations (Spearman correlations: ρ = 0.40 to ρ = 0.69). We concluded that, based on the results of this study, a cross-culturally validated Brazilian-Portuguese version of the Ped-ANAM has been developed and it is a reliable tool for the screening cognitive function.

International consensus statement on allergy and rhinology: Olfaction.

BACKGROUND: The literature regarding clinical olfaction, olfactory loss, and olfactory dysfunction has expanded rapidly over the past two decades, with an exponential rise in the past year. There is substantial variability in the quality of this literature and a need to consolidate and critically review the evidence. It is with that aim that we have gathered experts from around the world to produce this International Consensus on Allergy and Rhinology: Olfaction (ICAR:O). METHODS: Using previously described methodology, specific topics were developed relating to olfaction. Each topic was assigned a literature review, evidence-based review, or evidence-based review with recommendations format as dictated by available evidence and scope within the ICAR:O document. Following iterative reviews of each topic, the ICAR:O document was integrated and reviewed by all authors for final consensus. RESULTS: The ICAR:O document reviews nearly 100 separate topics within the realm of olfaction, including diagnosis, epidemiology, disease burden, diagnosis, testing, etiology, treatment, and associated pathologies. CONCLUSION: This critical review of the existing clinical olfaction literature provides much needed insight and clarity into the evaluation, diagnosis, and treatment of patients with olfactory dysfunction, while also clearly delineating gaps in our knowledge and evidence base that we should investigate further.

Competências profissionais para a atenção à saúde do trabalhador / Professional competencies for occupational health care

Resumo: Introdução: As competências profissionais para as ações em saúde direcionadas à população trabalhadora são imprescindíveis para a formação exitosa dos profissionais de saúde. Embora relevantes e bem definidas pela Política Nacional de Saúde do Trabalhador e da Trabalhadora, ainda existem lacunas sobre as competências mínimas requeridas e pouco conhecimento sobre como esses saberes profissionais se articulam para a promoção do trabalho integrado e o atendimento às necessidades dessa população. Objetivo: Este estudo teve como objetivo identificar as competências profissionais para atenção à saúde do trabalhador no contexto da APS. Método: Trata-se de pesquisa descritivo-exploratória, de abordagem qualitativa, desenvolvida por meio da triangulação de métodos, realizada em uma cidade do interior do estado de São Paulo. Os dados foram coletados em três etapas: 1. análise documental das Diretrizes Curriculares Nacionais dos cursos da saúde e dos projetos pedagógicos de sete cursos da área da saúde de uma instituição de ensino superior; 2. revisão sistemática da literatura; e 3. entrevistas com docentes dos cursos da saúde da referida instituição e com profissionais da Rede de Atenção Primária à Saúde municipal. Resultado: A análise documental permitiu identificar que somente os cursos de Educação Física, Enfermagem, Fisioterapia e Terapia Ocupacional possuem competências específicas para atenção à saúde do trabalhador, e a análise dos projetos pedagógicos apontou disciplinas obrigatórias e optativas que abordam a temática. A revisão de literatura e as entrevistas apontaram as competências gerais e específicas necessárias para atenção à saúde do trabalhador, como o cuidado integral, a compreensão dos determinantes sociais de saúde, a comunicação, a liderança, o trabalho em equipe, a gestão de conflitos, o acolhimento e a escuta qualificados, a gestão em saúde e a educação permanente. Conclusão: A identificação das competências profissionais gerais e específicas para atenção à saúde do trabalhador mostrou-se de extrema relevância para construção de uma assistência integral e humanizada na formação e prática interprofissional.

Abstract: Introduction: For health actions aimed at the working population, the development of professional competencies during the graduation is essential. Although relevant and well defined by the National Occupational Health Policy, there are still gaps regarding the minimum skills required and lack of knowledge about how the articulation of this competencies to promote integrated work and meet the needs of this population. Objective: This study aimed to identify the professional competencies for occupational health care. Method: Descriptive-exploratory research, with a qualitative approach, developed through the triangulation of methods, carried out in a city in the interior of the State of São Paulo, Brazil. Data were collected in three stages: 1. document analysis of the National Curriculum Guidelines for health courses and pedagogical projects for seven courses in the health area of a higher education institution; 2. systematic literature review; and 3. interviews with professors from the health courses of that institution and with professionals from the municipal Primary Health Care Network. Result: The document analysis allowed us to identify that only the Physical Education, Nursing, Physiotherapy and Occupational Therapy courses have specific competences for worker health care; the analysis of the pedagogical projects pointed to mandatory and optional subjects that address the theme. The literature review and interviews pointed out the general and specific skills needed for worker health care, such as comprehensive care, understanding the social determinants of health, communication, leadership, teamwork, conflict management, welcoming/qualified listening, health management and continuing education. Conclusion: The identification of general and specific professional skills for occupational health care proved to be extremely relevant for the construction of comprehensive and humanized care in training and interprofessional practice.

Consensus-driven conceptual development of a standardized whole body-MRI scoring system for assessment of disease activity in juvenile idiopathic arthritis: MRI in JIA OMERACT working group.

OBJECTIVES: Whole body-MRI is helpful in directing diagnostic and treatment approaches, and as a research outcome measure. We describe our initial consensus-driven phase towards developing a whole body-MRI scoring system for juvenile idiopathic arthritis. METHODS: An iterative approach using three rounds of anonymous Delphi surveys followed by a consensus meeting was used to draft the structure of the whole body-MRI scoring system, including the relevant anatomic joints and entheses for assessment, diagnostic item selection, definition and grading, and selection of appropriate MRI planes and sequences. The surveys were completed independently by an international expert group consisting of pediatric radiologists and rheumatologists. RESULTS: Twenty-two experts participated in at least one of three rounds of Delphi surveys and a concluding consensus meeting. A first iteration scoring system was developed which ultimately included the assessment of 100 peripheral, 23 chest, and 76 axial joints, and 64 entheses, with 2-4 diagnostic items graded in each of the items, using binary (presence/absence) and 2-3-level ordinal scores. Recommendations on anatomic MRI planes and sequences were specified as the minimally necessary imaging protocol for the scoring system. CONCLUSION: A novel whole body-MRI scoring system for juvenile idiopathic arthritis was developed by consensus among members of MRI in JIA OMERACT working group. Further iterative refinements, reliability testing, and responsiveness are warranted in upcoming studies.

The importance of transition from pediatric to adult rheumatology care in juvenile idiopathic arthritis.

Introduction: Juvenile idiopathic arthritis (JIA) is the most common rheumatic inflammatory condition in childhood. The long-term morbidity, mortality, and quality of life have improved with the earlier use of disease-modifying drugs (DMARDs) and the availability of biology disease-modifying drugs (bDMARDs). Despite the improvement of treatment, around 50% of the patients reach adulthood with articular and/or extra articular disease activity. A careful planned transition from pediatric to adult care is necessary to reduce the loss of follow-up that is associated with stopping medications, flares, and disability due to untreated arthritis or uveitis.Areas covered: This narrative review provides an overview of the importance of transition in JIA Articles were selected from Pubmed searches.Expert opinion: JIA patients, family, and healthcare workers have to be trained to provide an effective transition plan, based on local and national policies. Important aspects such as expectations, maturation, disease characteristics, disease activity, adherence, disability, and psychological aspects among others have to be considered and addressed during the transition phase to improve self-esteem, self-assurance, and quality of life.

Novos Tempos, Novos Desafios: Estratégias para Equidade de Acesso ao Ensino Remoto Emergencial / New Times, New Challenges: Strategies to Ensure Equal Access to Emergency Remote Education

Resumo: Introdução: Com a suspensão das atividades presenciais e a implementação do ensino remoto do curso de Medicina da Unicamp, foi necessária a elaboração de estratégias para identificar as necessidades dos estudantes para continuidade das aulas nessa nova modalidade. Objetivo: Demonstrar as estratégias desenvolvidas e as soluções encontradas para permitir a equidade de acesso ao ensino remoto no curso de medicina da FCM-Unicamp. Método: São apresentados dados do relatório institucional, elaborado a partir de questionários aplicados para os estudantes do curso de Medicina sobre as dificuldades referentes ao acesso ao ensino remoto emergencial e as ações realizadas para solucionar as principais dificuldades encontradas. Resultados: Constatamos que os anos iniciais do curso apresentam a maior proporção de alunos com dificuldades, embora, em todos os anos, os discentes tenham relatado alguma dificuldade. Os principais problemas identificados foram internet instável e/ou apenas acesso por redes móveis. Verificou-se ainda que os alunos tinham maior dificuldade em acompanhar as atividades síncronas. A maior parte dos alunos mencionou que acompanhava as aulas por computadores e notebooks, mas, muitas vezes, estes eram compartilhados com outros membros da família. Alguns estudantes apontaram também que muitas vezes, não conseguiam acompanhar o curso por falta de aparelhos ou acesso à internet. Nesse caso, computadores e chips de celular e para uso em tablets eram emprestados aos alunos. Houve orientação aos professores para adequação das ferramentas pedagógicas utilizadas. Conclusão: Garantir a equidade de acesso é fundamental para permitir a continuidade dos estudos na transformação do estudo presencial em remoto emergencial. O ato de escutar os alunos sobre as dificuldades em relação ao ensino remoto e a realização de suporte material são ferramentas essenciais para o sucesso dessa estratégia pedagógica. A orientação do corpo docente em relação às dificuldades dos alunos foi importante para adequação do ensino remoto. Essas ações auxiliaram os estudantes na mudança do ensino imposta pelo distanciamento social.

Abstract: Introduction: After the implementation of remote teaching for the academic activities of the Medicine course at UNICAMP, due to the COVID Pandemic, a new strategy was required to identify the needs of students to allow them to keep up with the course in this new pedagogical modality. Objective: To demonstrate the strategies developed and solutions found to allow for equal access to remote education in the medical course at FCM-UNICAMP. Method: We present data from our institutional report based on questionnaires completed by medical students regarding the difficulties related to access to emergency remote education and the actions taken to solve the main difficulties encountered. Results: We identified that the initial years of the course had the highest proportion of students with difficulties, although all students reported some difficulty. The main problems identified were unstable internet and/or only access via mobile networks, and the most significant difficulty was in accessing the synchronous activities. Most students reported attending classes via computer and notebooks, but this device was often shared with other family members, most notably during the pandemic. Thirty-one students reported difficulties that prevented them from following the course adequately due to lack of devices or internet access. One solution was computers and cell phone chips and tablets being loaned out to students. Teachers were instructed to adapt the pedagogical tools used. Conclusion: Ensuring equal access is essential to support studies to continue following the transition from face-to-face classes to emergency remote study. Listening to students about the difficulties concerning remote teaching is an essential tool for the success of this pedagogical strategy. Guidance of the teaching staff in relation to the difficulties encountered by students was important for the adequacy of remote teaching. These actions helped students in the change of teaching imposed by social distancing.

MiniCex as an Assessment Tool of a Medical Course Internship Program / MiniCex como Instrumento para Avaliação de Programa no Internato de um Curso de Medicina / MiniCex como Instrumento para Avaliação de Programa no Internato de um Curso de Medicina

Abstract: Introduction: Program assessment is the process of data collection about a course or teaching program that takes into consideration the aspects of cost-effectiveness, checking the adequacy of the evaluation according to the course purpose and the program capacity to yield changes in real life. Such regular assessments provide feedback to the decision-making process that aim at better teaching and learning practices. The Mini Clinical Evaluation Exercise (MiniCex) is a performance rating scale designed to assess the skills that medical students and residents need in real-life situations with patients. Considering the importance of program assessment for an institution, the utilization of the MiniCex data might be of great value for the follow-up of students and the course, helping the planning process and generating improvements in the institution. Therefore, the objective of this study is to assess the program using MiniCex in the beginning of the medical internship, aiming to determine in what areas of the basic and pre-clinical course the students have more difficulties. Methods: A cross-sectional descriptive study was carried out, using the retrospective data obtained by the MiniCex forms that were applied to the 9th-semester medical students, which correspond to the first semester of medical internship in the Federal University of Pará. A total of 111 students was assessed, among the 154 students eligible for internship, from August 2017 to July 2018. Results: Among the performed evaluations, with 97% being requested by the teachers, most of them (72%) were about new cases, and 45% and 38% had low or moderate complexity, respectively. There was a predominance of musculoskeletal system disorders (27.7%), followed by the gastrointestinal/hepatology system (14.8%). Concerning the skills in each domain, the performance was satisfactory in all of them. We observed that 12% of the students had difficulties in at least one area, followed by 6.3% of students with difficulties in 2 areas and 4.5% with an unsatisfactory performance in 3 or more areas. Conclusion: the MiniCex, when applied to internship students, showed to be a source of important and useful information, as part of a program assessment concerning the areas preceding the internship. The analysis of the obtained data was sent to teachers of the pre-internship, internship and course management areas. To the first ones, with the objective of reviewing their programs, detecting where they can intervene and, thus, make changes that aim a better acquisition of basic knowledge by the students and, consequently, improve their performance. To the second ones, to provide an overview of where they will have to focus their programs according to the needs of the medical students who reach the internship. Finally, to the course management, as a guide of what should be supervised by the professionals teaching the semesters that precede the internship.

Resumo: Introdução: avaliação de programa é o processo de obtenção de informações sobre um curso ou programa de ensino que leva em consideração aspectos de custo-efetividade, de checagem da adequação da avaliação ao propósito do curso e da capacidade do programa de induzir transformação da realidade. Tais avaliações regulares retroalimentam as tomadas de decisão que almejam melhores práticas de ensino e aprendizagem. O Miniexecício Clínico Avaliativo (Mini Clinical Evaluation Exercise - MiniCex) é uma escala de classificação de desempenho projetada para avaliar as habilidades que os acadêmicos e residentes necessitam em encontros reais com os pacientes. Diante da importância da avaliação de programa para uma instituição, a utilização de dados do MiniCex pode ser de grande valia para o acompanhamento dos alunos e do curso, favorecendo o planejamento e as melhorias na instituição. Objetivo: utilizar o MiniCex como parte de uma avaliação de programa no início do internato do curso de Medicina, visando determinar as áreas do curso básico e pré-clínico nas quais o aluno possui deficiências. Métodos: Foi realizado um estudo transversal, de caráter descritivo, com a utilização de dados retrospectivos obtidos por meio das fichas do MiniCex aplicadas aos alunos do nono semestre no módulo de Clínica Médica que correspondeu ao primeiro semestre do internato da Faculdade de Medicina da Universidade Federal do Pará, sendo avaliados um total de 111 alunos dentre os 154 aptos ao internato no período de agosto de 2017 a julho de 2018. Resultados: Dentre as avaliações realizadas, com 97,2% solicitadas pelos professores, a maioria (72%) foi de casos novos, 45% e 38,7% de baixa e moderada complexidade, respectivamente. Houve predomínio afecções do sistema musculoesquelético (27,7%), seguido do sistema gastrointestinal/hepatologia (14,8%). Quanto às habilidades em cada domínio, obteve-se rendimento suficiente em todos. Observou-se que 12,6% dos alunos tiveram deficiência em pelo menos uma área, o que foi seguido de 6,3% de alunos insuficientes em duas áreas e 4,5% com rendimento insatisfatório em três ou mais áreas. Conclusão: o MiniCex aplicado aos estudantes do internato mostrou-se capaz de fornecer informações importantes e úteis como parte de uma avaliação de programa das áreas prévias ao internato. A análise dos dados obtidos foi encaminhada aos professores do pré-internato e do internato e à direção do curso. Enviou-se a análise aos primeiros para que pudessem rever seus programas e detectar em que ponto podem intervir e fazer as alterações que visem à melhor aquisição de conhecimentos básicos pelos discentes e consequentemente ao aumento do desempenho deles. Quanto aos professores do internato, o objetivo foi apresentar-lhes um panorama dos aspectos em que precisarão concentrar seus programas conforme as carências indicadas pelos acadêmicos que chegam ao internato. Por último, à direção, o material serviu de guia do que deve fiscalizar dos docentes dos semestres que antecedem o internato.

Resumo: Introdução: avaliação de programa é o processo de obtenção de informações sobre um curso ou programa de ensino que leva em consideração aspectos de custo-efetividade, de checagem da adequação da avaliação ao propósito do curso e da capacidade do programa de induzir transformação da realidade. Tais avaliações regulares retroalimentam as tomadas de decisão que almejam melhores práticas de ensino e aprendizagem. O Miniexecício Clínico Avaliativo (Mini Clinical Evaluation Exercise - MiniCex) é uma escala de classificação de desempenho projetada para avaliar as habilidades que os acadêmicos e residentes necessitam em encontros reais com os pacientes. Diante da importância da avaliação de programa para uma instituição, a utilização de dados do MiniCex pode ser de grande valia para o acompanhamento dos alunos e do curso, favorecendo o planejamento e as melhorias na instituição. Objetivo: utilizar o MiniCex como parte de uma avaliação de programa no início do internato do curso de Medicina, visando determinar as áreas do curso básico e pré-clínico nas quais o aluno possui deficiências. Métodos: Foi realizado um estudo transversal, de caráter descritivo, com a utilização de dados retrospectivos obtidos por meio das fichas do MiniCex aplicadas aos alunos do nono semestre no módulo de Clínica Médica que correspondeu ao primeiro semestre do internato da Faculdade de Medicina da Universidade Federal do Pará, sendo avaliados um total de 111 alunos dentre os 154 aptos ao internato no período de agosto de 2017 a julho de 2018. Resultados: Dentre as avaliações realizadas, com 97,2% solicitadas pelos professores, a maioria (72%) foi de casos novos, 45% e 38,7% de baixa e moderada complexidade, respectivamente. Houve predomínio afecções do sistema musculoesquelético (27,7%), seguido do sistema gastrointestinal/hepatologia (14,8%). Quanto às habilidades em cada domínio, obteve-se rendimento suficiente em todos. Observou-se que 12,6% dos alunos tiveram deficiência em pelo menos uma área, o que foi seguido de 6,3% de alunos insuficientes em duas áreas e 4,5% com rendimento insatisfatório em três ou mais áreas. Conclusão: o MiniCex aplicado aos estudantes do internato mostrou-se capaz de fornecer informações importantes e úteis como parte de uma avaliação de programa das áreas prévias ao internato. A análise dos dados obtidos foi encaminhada aos professores do pré-internato e do internato e à direção do curso. Enviou-se a análise aos primeiros para que pudessem rever seus programas e detectar em que ponto podem intervir e fazer as alterações que visem à melhor aquisição de conhecimentos básicos pelos discentes e consequentemente ao aumento do desempenho deles. Quanto aos professores do internato, o objetivo foi apresentar-lhes um panorama dos aspectos em que precisarão concentrar seus programas conforme as carências indicadas pelos acadêmicos que chegam ao internato. Por último, à direção, o material serviu de guia do que deve fiscalizar dos docentes dos semestres que antecedem o internato.

Syndromic Oral Clefts: Challenges of Genetic Assessment in Brazil and Suggestions to Improve Health Policies.

Addressing the unmet health needs of persons living with congenital anomalies in low- and middle-income countries (LMIC) is a major challenge. Registries and databases are exemplary tools capable to link research data with health programs. Since 2009, Brazil's Craniofacial Project, a multicenter and voluntary research initiative, collects socioeconomic, medical, and genetic information on individuals with craniofacial anomalies through the Brazilian Database on Craniofacial Anomalies (BDCA). This article discusses challenges to the provision of genetic assessment and counselling for individuals with syndromic oral clefts (SOC) through public health services in LMIC, such as Brazil. Subjects were selected using methods of the BDCA as described elsewhere. Among 800 records, 66 assigned as SOC with no etiologic diagnosis were preselected for genomic imbalance screening. Only 28 have timely completed basic protocol using public health services, and 22 were able to perform chromosomal microarray analysis. Pathogenic genomic imbalances were identified in 4 (18.18%) and a copy number variation of uncertain clinical significance was detected in one. Results exemplify barriers faced by the majority of the population of Brazil to reach whole genetic assessment either through public genetic services or in research settings. In this unfavorable scenario, BDCA has allowed the recognition of individuals with similar needs, optimizing the scarce genetic laboratory facilities in Brazil. Ultimately, BDCA has facilitated the translation of research into care. This experience may be successfully extended to other congenital anomalies and to LMIC with similar characteristics. A set of suggestions focusing on oral clefts is provided.

Assessment of Bone Mineral Density of Patients with Spinocerebellar Ataxia Type 3.

OBJECTIVE: Machado-Joseph disease (MJD) is a spinocerebellar ataxia, and osteoporosis is a multifactor disease that may affect patients with neurologic conditions. The frequency of osteoporosis among MJD patients, however, has not been studied. The purpose of this study is to evaluate bone mineral density (BMD) and identify correlations between clinical factors and frequency of vertebral fractures in patients with MJD. METHODS: Clinical data, lumbar X-rays and BMD data were obtained in 30 patients with MJD. RESULTS: Ten patients (33.3%) showed low BMD in at least one of the sites studied based on Z-scores. The Z-score correlated directly with body mass index, and the femoral neck Z-score was inversely correlated with cytosine-adenine-guanine (CAG) expansion. There was no correlation between BMD and other clinical factors. Forty-three percent of the patients reported previous pathologic fractures. Five patients (16.7%) had at least one fracture detected by lumbar X-ray. CONCLUSION: Low BMD and fractures are frequent among MJD patients, and careful management of BMD may be beneficial for these patients.

Assessment of the Hospital Anxiety and Depression Scale (HADS) performance for the diagnosis of anxiety in patients with systemic lupus erythematosus.

Neuropsychiatric lupus (NPSLE) is described in 12-95% of patients with systemic lupus erythematosus (SLE). Anxiety disorders are among the most frequent manifestations of NPSLE, occurring in 4-85% of these patients. Several diagnostic tools, such as Hospital Anxiety and Depression Scale (HADS), have been used to assess anxiety in clinical studies in SLE, but there is a lack of data on the performance of these questionnaires in the disease. This study aimed to assess the performance of HADS for the detection of anxiety in male and female patients with SLE, also investigating possible gender differences in this aspect. This study included 54 male SLE patients and 54 female SLE patients. The Diagnostic Criteria for Generalized Anxiety Disorder of the 5th edition of the Diagnostic and Statistical Manual of Mental Disorders was used as gold-standard method to assess the performance of HADS for detecting anxiety in SLE patients. HADS presented sensitivity of 88.9% and specificity of 92.6%, with positive and negative predictive values of 80.0 and 96.1%, respectively. The HADS accuracy in total sample was 92.6%, with Kappa coefficient equal to 0.5794 (95% CI 0.3894-0.7695). No significant differences were observed between female and male groups regarding the performance of HADS for diagnosing anxiety.

Assessment of condyle, masseter and temporal muscles volumes in patients with juvenile systemic lupus erythematosus: A cross-sectional study.

AIMS: The purpose of this study was to evaluate the effect of the corticosteroid therapy for both treatment of juvenile systemic lupus erythematosus and disease activity on two masticatory muscles and condyle of the temporomandibular joint. METHODS: A total of 21 controls and 48 juvenile systemic lupus erythematosus patients were investigated. Volumes of the temporal and masseter muscles and condyle of the subjects were assessed by using a 3D reconstruction in magnetic resonance imaging. The ITK-SNAP, a medical imaging software, was used for 3D reconstruction. A dental examination with registration of occlusion was performed in subjects. Data were statistically analyzed by means of the Dahlberg's test associated with paired t-test, Fisher's exact test and Chi-square. RESULTS: There was a positive correlation between temporalis muscle and age (p = 0.032), masseter volume (p = 0.029) and condyle volume (p = 0.013). The mean volume measurements of temporal and masseter muscles and condyle were not statistically associated with juvenile systemic lupus erythematosus regarding disease activity and corticosteroid therapy (p > 0.05). There were no significant differences between malocclusion and volume of muscles and condyle. CONCLUSION: This study suggested that volume of the target structures has no correlation with cumulative corticosteroid dose, disease activity, and malocclusion.

Neuropsychiatric manifestations in systemic lupus erythematosus: epidemiology, pathophysiology and management.

Systemic lupus erythematosus (SLE) is a relapsing-remitting autoimmune disease with CNS involvement occurring in up to 75% of patients. However, the frequency of neuropsychiatric manifestations in SLE studies varies widely, depending on the type of manifestations included and the method used for evaluation. CNS involvement may be considered primary if directly related to SLE activity in the CNS or secondary when related to treatment, infections, metabolic abnormalities or other systemic manifestations such as uraemia and hypertension. The pathogenesis of neuropsychiatric SLE is as yet unknown, though numerous autoantibodies and cytokines have been suggested as possible mediators. However, independent of the aetiology of the insult, the final common pathway in neuropsychiatric SLE is the involvement of the cerebral microvasculature. The diagnosis of primary CNS involvement by SLE is often difficult, as both focal and diffuse manifestations may occur and there is no gold standard for diagnosis. A high index of clinical suspicion, in addition to laboratory and neuroimaging findings may support the diagnosis. Treatment is mostly empirical, although one randomized controlled trial has shown that cyclophosphamide in addition to methylprednisolone is superior to methylprednisolone alone in severe neuropsychiatric SLE.