Evaluation of the responsiviness of three instruments of health related quality of life: prospective study in patients with systemic lupus erythematosus

I- Introdução: O parâmetro qualidade de vida tem sido cada vez mais considerado na avaliação de doenças crônicas. Na língua portuguesa não existem instrumentos de avaliação de Qualidade de vida específicos para Lupus Eritematoso Sistêmico(LES). II- Objetivo: Traduzir para o português, fazer adaptação cultural e avaliar a confiabilidade, validade e responsividade dos questionários Systemic Lupus Erythematosus Quality of Life (SLEQOL) e Systemic Lupus Erythematosus Symptom Checklist (SSC) e também avaliar a responsividade do Medical Outcomes Study 36 –Item Short-Form Health Survey- (SF-36),. III- Pacientes e Métodos: População estudada: 107 pacientes com LES de acordo com os critérios do ACR, maiores que 16 anos , responderam ao SLEQOL, SSC e SF-36 , durante 4 visitas ao longo de 1 ano, no intuito de tradução dos instrumentos e de verificarmos as suas propriedades psicométricas.Tradução: Tradução e adaptação cultural para língua portuguesa dos questionários de acordo com os trabalhos que abordam a metodologia de tradução de questionário para outros idiomas após a autorização dos respectivos autores. A confiabilidade foi analisada através de 3 entrevistas com entrevistadores diferentes, sendo 2 no mesmo dia (inter-observador) e a terceira até 14 dias após a primeira avaliação (intra-observador). A validade foi avaliada através das correlações entre parâmetros clínicos e de Qualidade de vida com SLEQOLeSSC. A responsividade foi determinada em cada questionário e em cada domínio de duas maneiras: Através do método baseado em âncoras e do Método baseado em características estatísticas da variação da amostra. Análise estatística: Análise descritiva para caracterização da amostra. Coeficiente de correlação intraclasse (CCI) para avaliação da reprodutibilidade intra e inter-observadoress A consistência interna dos itens foi avaliada por meio do coeficiente alfa de Cronbach . Para estudo da validade utilizamos o coeficiente de correlação de Pearson. Para avaliação da responsividade utilizamos o teste estatístico t para avaliação das mudanças nos escores como também calculamos o tamanho do efeito (effect size) destas mudanças entre cada visita no intuito de detectarmos a diferença mínima clinicamente importante(DMCI). Para todos os testes estatísticos, o nível de significância adotado foi de 5%. IV- Resultados: Foram avaliados 107 pacientes com LES em 4 visitas trimestrais. Os questionários SLEQOL e SSC foram traduzidos após concordância dos autores. Feitas as traduções, traduções de volta (back translation) e adaptação cultural. Os principais resultados obtidos para o SLEQOL foram: o coeficiente de correlação obtido da consistência interna foi de 0,807 para todas as questões e também por domínios e os coeficientes de correlação inter-e intra-observadores foram respectivamente de 0,990 e 0,969. Os coeficientes de correlação para consistência interna, de reprodutibilidade intra-observador e reprodutibilidade inter-observador para o SSC foi 0,874, 0,925 e 0,917 respectivamente. Na validação tanto de SLEQOL como de SSC houve moderada correlação com SF-36, porém baixa correlação com atividade e dano da doença. Na responsividade alcançamos a DMCI em alguns domínios de SLEQOL e SF-36 como também em SSC principalmente através do método baseado em âncoras.V- Conclusões: SLEQOL e SSC são questionários, abrangentes, de fácil aplicação e o SLEQOL além de sintomas físicos avaliam bem o estado mental e o bem estar dos pacientes. São psicometricamente robustos demonstrando reprodutibilidade, validade e responsividade ao longo do tempo, principalmente quando usamos o método de âncoras como opinião do paciente e índice de atividade de doença, além de expressar a opinião do paciente fator importante no acompanhamento da doença.I- Introduction: The quality of life parameter has been increasingly considered in the assessment of chronic disease. There are no quality of life assessment instruments specific to Systemic Lupus Erythematosus in the Portuguese language. II- Objective: Translate into Portuguese, cross-culturally adapt and assess the reliability, validity and responsiveness of the Systemic Lupus Erythematosus Quality of Life (SLEQOL) and Systemic Lupus Erythematosus Symptom Checklist (SSC) questionnaires as well as assess the responsiveness of the Medical Outcomes Study 36- Item Short-Form Health Survey (SF-36). III- Patients and Methods: Population studied: 107 patients with lupus (according to ACR criteria) over 16 years of age responded to the SLEQOL, SSC and SF-36 during four visits over the course of one year with the aim of translating the instruments and determining their psychometric properties. Translation and cross-cultural adaptation of the questionnaires into the Portuguese language was performed in accordance with studies addressing questionnaire translation methodology following the authorization of the respective authors. Reliability was analyzed through three interviews with different interviewers: two on the same day (inter-observer) and a third interview conducted by one of the interviewers within 14 days of the first evaluation (intra-observer). Validity was assessed through the correlations between clinical and quality of life parameters as assessed by the SLEQOL and SSC. Responsiveness was determined for each questionnaire and each domain in two manners: Through the anchor-based method and the method based on statistical characteristics of variation in the sample. Statistical analysis: Descriptive analysis was performed for the characterization of the sample. The intra-class correlation coefficient was used to assess intra-observer and inter-observer reproducibility. Internal consistency of the items was assessed using Cronbach’s alpha coefficient. Pearson’s correlation coefficient was used to study validity. For the assessment of responsiveness, the Student’s t-test was used to assess changes in the scores; we also calculated the effect size of these changes between each visit in order to detect the minimal clinically important difference (MCID). The significance level for all statistical tests was set at 5 percent. IV- Results: 107 patients with lupus were evaluated in four trimestral visits. The SLEQOL and SSC questionnaires were translated following authorization from the authors. Following the translations, back translation and cross-cultural adaptation were performed. The main results obtained for the SLEQOL were a 0.807 correlation coefficient for internal consistency for all questions and domains as well as a 0.990 inter-observer and 0.969 intra-observer correlation coefficient. The correlation coefficients for internal consistency, intra-observer reproducibility and inter-observer reproducibility for the SSC were 0.874, 0.925 and 0.917, respectively. In the validation, both the SLEQOL and the SSC demonstrated a moderate correlation with the SF-36, but a low correlation with disease activity and damage. Regarding responsiveness, we achieved a MCID in some SLEQOL and SF-36 domains as well as the SSC, mainly through the anchor-based method. V- Conclusions: The SLEQOL and SSC are broad-based questionnaires of easy application. The SLEQOL performs well in assessing physical symptoms and the mental status and wellbeing of patients. Both instruments are robust, demonstrating reproducibility, validity and responsiveness over time, especially when we used the anchor-based method for the opinion of patients and the disease activity index, as well as expressing the opinion of the patient, which is an important factor in the follow up of the disease.Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)BV UNIFESP: Teses e dissertaçõe

DNMT3B (rs2424913) polymorphism is associated with systemic lupus erythematosus alone and with co-existing periodontitis in a Brazilian population

The association between Periodontitis and Systemic Lupus Erythematosus (SLE) has been primarily based on their similar pathophysiology and both are associated with genetic polymorphisms. Objectives: To investigate an association between the methylation-related gene polymorphisms DNMT3B (rs2424913) and MTHFR (rs1801133) to Systemic Lupus Erythematosus (SLE) and Periodontitis. Methodology: In total, 196 individuals of all genders aged 24 to 60 years old were allocated into four groups based on their systemic and periodontal status, namely: Healthy control (n=60), periodontitis (n=51), SLE (n=47), and SLE + periodontitis (n=38). Individuals with SLE were stratified according to disease activity (SLEDAI) in inactive or active. We performed polymorphism analysis using PCR-RFLP with genomic DNA from mouthwash. We analyzed data using Fisher’s Exact, Chi-square test, and regression models. Results: Periodontal status were similar in subjects with periodontitis alone and combined with SLE. SLE patients with periodontitis had a longer SLE diagnosis than SLE only (p=0.001). For DNMT3 B polymorphism, the periodontitis, SLE, and Inactive SLE + periodontitis groups showed a higher frequency of T allele and TT genotypes compared to healthy controls (p<0.05). Regression analyses showed that the TT genotype is a strong risk factor for periodontitis (OR=4.53; CI95%=1.13–18.05) and also for SLE without periodontitis (OR=11.57; CI95%=3.12–42.84) and SLE with periodontitis (OR=5.27; CI95%=1.25–22.11) when compared to control. Conclusion: SLE patients with periodontitis had a longer length of SLE diagnosis. The DNMT3B (rs2424913) polymorphism was associated with periodontitis and SLE alone or combined with periodontitis. Our study contributes to understanding the genetic mechanisms involved in periodontitis and SLE susceptibility

Brazilian recommendations on the safety and effectiveness of the yellow fever vaccination in patients with chronic immune-mediated inflammatory diseases

Background: In Brazil, we are facing an alarming epidemic scenario of Yellow fever (YF), which is reaching the most populous areas of the country in unvaccinated people. Vaccination is the only effective tool to prevent YF. In special situations, such as patients with chronic immune-mediated inflammatory diseases (CIMID), undergoing immunosuppressive therapy, as a higher risk of severe adverse events may occur, assessment of the risk-benefit ratio of the yellow fever vaccine (YFV) should be performed on an individual level. Main body of the abstract: Faced with the scarcity of specific orientation on YFV for this special group of patients, the Brazilian Rheumatology Society (BRS) endorsed a project aiming the development of individualized YFV recommendations for patients with CIMID, guided by questions addressed by both medical professionals and patients, followed an internationally validated methodology (GIN-McMaster Guideline Development). Firstly, a systematic review was carried out and an expert panel formed to take part of the decision process, comprising BRS clinical practitioners, as well as individuals from the Brazilian Dermatology Society (BDS), Brazilian Inflammatory Bowel Diseases Study Group (GEDIIB), and specialists on infectious diseases and vaccination (from Tropical Medicine, Infectious Diseases and Immunizations National Societies); in addition, two representatives of patient groups were included as members of the panel. When the quality of the evidence was low or there was a lack of evidence to determine the recommendations, the decisions were based on the expert opinion panel and a Delphi approach was performed. A recommendation was accepted upon achieving ≥80% agreement among the panel, including the patient representatives. As a result, eight recommendations were developed regarding the safety of YFV in patients with CIMID, considering the immunosuppression degree conferred by the treatment used. It was not possible to establish recommendations on the effectiveness of YFV in these patients as there is no consistent evidence to support these recommendations. Conclusion: This paper approaches a real need, assessed by clinicians and patient care groups, to address specific questions on the management of YFV in patients with CIMID living or traveling to YF endemic areas, involving specialists from many areas together with patients, and might have global applicability, contributing to and supporting vaccination practices. We recommended a shared decision-making approach on taking or not the YFV

QUALITY OF LIFE IN SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS IN NORTHEASTERN BRAZIL: IS HEALTH-RELATED QUALITY OF LIFE A PREDICTOR OF SURVIVAL FOR THESE PATIENTS?

Objective: To identify social, demographic and clinical characteristics that influence survival of patients with systemic lupus erythematosus (SLE). Methods: Sixty-three patients with a diagnosis of SLE were studied at our medical services in 1999 and then reviewed in 2005. We utilized a protocol to obtain demographic and clinical traits, activity and damage indices, and health-related quality of life via the SF-36. All statistical tests were performed using a significance level of 5%. Results: Out of the 63 patients examined in 1999, six died, four were lost for the follow-up and the previous protocol was applied to the remaining 53 patients. The six patients who died presented the worst recorded health-related quality of fife, in all aspects. The most important observed predictor of death was a mean lower score in the Role-Emotional Domain of the mental health component of the SF-36 (p<0.01). Conclusion: Health-related quality of life may be used as possible predictive factor of mortality among patients with SLE

Tradução, adaptação cultural e validação para a língua portuguesa (Brasil) do Systemic Sclerosis Questionnaire (SySQ)

Introdução:A esclerose sistêmica (ES) é uma doença multissistêmica, autoimune, caracterizada por disfunção fibroblástica e vasculopatia, causando grande impacto na qualidade de vida (QV). Esta é mensurada por instrumentos ou questionários, geralmente formulados em outros idiomas e inseridos em contextos culturais distintos.Objetivo:Traduzir, adaptar culturalmente e validar para a língua portuguesa (Brasil) o questionário do Systemic Sclerosis Questionnaire (SySQ) de QV em ES.Metodologia:Tradução e adaptação: etapa realizada de acordo com metodologia específica de tradução de questionários. Confiabilidade: foi analisada através de três entrevistas, realizadas por diferentes entrevistadores, sendo duas no mesmo dia (interobservação) e uma terceira após 14 dias (intraobservação). Validade: avaliada pela correlação clínica e parâmetros de QV com os domínios do Sysc. Análise estatística: realizada análise descritiva da amostra. A reprodutibilidade foi avaliada através de um coeficiente de correlação intraclasse (ICC) e a consistência interna pelo coeficiente alfa de Cronbach, já para analisar a validade utilizou o coeficiente de correlação de Spearman.Resultados: Foram observados 16 pacientes portadores de ES. Os nossos resultados foram semelhantes aos do questionário original, com a consistência interna variando entre 0,73 e 0,93 para cada item. A reprodutibilidade interobservador foi muito boa para todos os domínios (&#945; = 0,786 a 0,983), e a intraobservador foi muito boa para o domínio de sintomas gerais (CCI = 0,916), boa para os domínios de sintomas musculoesqueléticos (CCI = 0,897) e cardiopulmonares (CCI = 0,842) e razoáveis para o de sintomas gastrintestinais (CCI = 0,686).Conclusão: A versão na língua portuguesa do SySQ mostrou-se reprodutível e válida para nossa população através de metodologia reconhecida para tradução e adaptação cultural de questionários

Análise dos critérios diagnósticos, de classificação, atividade e gravidade de doença na esclerose sistêmica Analysis of diagnostic, classification, activity and severity criteria in systemic sclerosis

Os autores analisam os critérios diagnósticos e de classificação da esclerose sistêmica (ES), as formas clínicas difusa e limitada, e os conceitos recentes da ES sine escleroderma e da ES precoce. Também discutem as limitações no estabelecimento dos critérios de atividade e gravidade da doença, e as recentes tentativas de padronização dos mesmos.<br>The authors analyze the diagnostic and classification criteria in systemic sclerosis (SSc), the clinical variants diffuse and limited, and the recent concepts of SSc sine scleroderma and early SSc. It is also discussed the limitations in the validation of the methods to measure SSc activity and damage, and the recent efforts in order to standardize them