Climatério e lúpus eritematoso sistêmico / Climacteric and systemic lupus erythematosus

O lúpus eritematoso sistêmico é uma doença crônica, complexa e multifatorial que apresenta manifestações em vários órgãos. O seu acometimento ocorre 10 vezes mais no sexo feminino do que no masculino. É uma doença com uma clínica variada e com graus variados de gravidade, causando fadiga, manifestações cutâneas, como rash malar, fotossensibilidade, queda de cabelo e manifestações musculoesqueléticas, como artralgia, mialgia e atrite. Podem ocorrer flares (crises), que se caracterizam por aumento mensurável na atividade da doença. No climatério, no período da pré-menopausa, o lúpus eritematoso sistêmico ocorre com mais frequência, podendo ocorrer também na pós-menopausa. Algumas doenças são mais frequentes na fase do climatério, e a presença do lúpus pode influenciar na sua evolução, como a doença cardiovascular, osteoporose e tromboembolismo venoso. A terapia hormonal oral determina aumento do risco de tromboembolismo venoso no climatério, e na paciente com lúpus eritematoso sistêmico há aumento dos riscos de flares e de trombose. Em vista disso, a terapia hormonal é recomendada apenas para pacientes com lúpus eritematoso sistêmico estável ou inativo, sem história de síndrome antifosfolípides e com anticorpos antifosfolípides negativa, devendo-se dar preferência para a terapia estrogênica transdérmica, em menor dose e de uso contínuo. Na paciente com lúpus eritematoso sistêmico ativo ou com história de síndrome antifosfolípides ou com anticorpos antifosfolípides positiva, recomenda-se a terapia não hormonal, como os antidepressivos. (AU)

Systemic lupus erythematosus is a chronic, complex, multifactorial disease that manifests in several organs. Its involvement occurs 10 times more in females than in males. It is a disease with a varied clinic and varying degrees of severity, causing fatigue, skin manifestations such as malar rash, photosensitivity, hair loss and musculoskeletal manifestations such as arthralgia, myalgia and arthritis. Flare may occur, which are characterized by measurable increase in disease activity. In the climacteric, in the premenopausal period, systemic lupus erythematosus occurs more frequently, and may also occur in the postmenopausal period. Some diseases are more frequent in the Climacteric phase and the presence of lupus can influence its evolution, such as cardiovascular disease, osteoporosis and venous thromboembolism. Oral hormone therapy determines an increased risk of venous thromboembolism in the climacteric and in patients with systemic lupus erythematosus there is an increased risk of flares and thrombosis. In view of this, hormone therapy is only recommended for patients with stable or inactive systemic lupus erythematosus, without a history of antiphospholipid syndrome and with antiphospholipid antibodies, giving preference to transdermal estrogen therapy, at a lower dose and for continuous use. In patients with active systemic lupus erythematosus or with a history of antiphospholipid syndrome or positive antiphospholipid antibodies, non-hormonal therapy, such as antidepressants, is recommended. (AU)

Sífilis mimetizando lúpus eritematoso sistêmico: um relato de caso / Syphilis mimicking systemic lupus erythematosus: a case report / Sífilis que simula lupus eritematoso sistémico: informe de un caso

O Lúpus Eritematoso Sistêmico (LES) é uma patologia crônica, de origem autoimune e inflamatória. As diversas manifestações clínicas existentes em pacientes acometidos pelo LES, sejam elas sistêmicas ou órgãos-alvo, possibilitam variados diagnósticos diferenciais. Dentre as manifestações clínicas que possibilitam estes diagnósticos está o acometimento cutâneo, com vasta variabilidade de apresentação. Da mesma forma, a sífilis também possui apresentação cutânea, tornando possível o diferencial de diagnóstico com outras patologias, inclusive o próprio LES. O presente estudo tem como objetivo relatar um caso de sífilis mimetizando lúpus eritematoso sistêmico, descrever o quadro clínico apresentado pelo paciente, bem como as ferramentas utilizadas para diagnóstico, e a posterior abordagem terapêutica. O caso relatado refere-se a um paciente de 29 anos, do sexo masculino, procedente de Campos Novos (SC), que apresentou um quadro clínico e laboratorial de lúpus-like induzido por uma infecção aguda de sífilis. A resolução completa de critérios inflamatórios de LES ocorreu após tratamento correto da doença infecciosa, com total melhora clínica e sorológica.

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease. The various clinical manifestations in SLE patients, both systemic and in target organs, allow for various differential diagnoses. Among the clinical manifestations that aid in diagnosis are the cutaneous injuries, which have a wide range of presentations. Syphilis also has cutaneous manifestations, which aid in the differential diagnosis from other pathologies, including SLE. The present study aims to report a case of syphilis mimicking SLE, describe the clinical condition presented by the patient, the tools used for diagnosis, and the therapeutic approach. The case reported refers to a 29- year-old male patient from Campos Novos (SC), who showed a clinical and laboratory lupus-like condition induced by an acute syphilis infection. The full resolution of SLE inflammatory criteria occurred following appropriate treatment for the infectious disease, with complete clinical and serological improvement.

El lupus eritematoso sistémico (LES) es una enfermedad inflamatoria autoinmune crónica. Las diversas manifestaciones clínicas de los pacientes con LES, tanto sistémicas como en órganos diana, permiten realizar varios diagnósticos diferenciales. Entre las manifestaciones clínicas que ayudan al diagnóstico se encuentran las lesiones cutáneas, que tienen una amplia gama de presentaciones. La sífilis también tiene manifestaciones cutáneas, que ayudan al diagnóstico diferencial con otras patologías, incluido el LES. El presente estudio tiene como objetivo comunicar un caso de sífilis que simula un LES, describir el cuadro clínico presentado por la paciente, las herramientas utilizadas para el diagnóstico y el abordaje terapéutico. El caso relatado se refiere a un paciente masculino de 29 años, natural de Campos Novos (SC), que presentó un cuadro clínico y de laboratorio semejante al lupus, inducido por una infección aguda por sífilis. La resolución completa de los criterios inflamatorios del LES ocurrió tras el tratamiento adecuado de la enfermedad infecciosa, con mejoría clínica y serológica completa.

Lupus Eritematoso Sistémico y Manifestaciones en el Sistema Nervioso Central: Un desafío diagnóstico permanente: casos Clínicos / Systemic lupus erythematosus and manifestations in the Central Nervous System: a permanent diagnostic challenge. Clinical cases

La presencia de manifestaciones neuropsiquiátricas en pacientes reumatológicos trae consigo un gran desafío diagnóstico que exige una mirada amplia, desde las bases de la medicina interna, a fin de poder orientar un estudio adecuado y el tratamiento oportuno. Junto con ello, el permanente diálogo e intercambio de miradas clínicas con otras especialidades permite tener un enfoque multidisciplinario que enriquece el abordaje de estas presentaciones complejas.

The presence of neuropsychiatric manifestations in rheumatological patients brings with it a great diagnostic challenge that requires a broad view, from the foundations of internal medicine, in order to guide the appropriate study and timely treatment of these patients. Along with this, the permanent dialogue and exchange of clinical views with other specialties allows for a multidisciplinary approach that enriches the approach to these complex presentations.

Cambio de diagnóstico y de categoría diagnóstica en pacientes con artritis idiopática juvenil en 7 años de seguimiento / Change of diagnosis and diagnostic category in patients with juvenile idiopathic arthritis within 7 years of follow-up

Resumen: Introducción: Al menos 50% de los pacientes pediátricos portadores de artritis idiopática juvenil (AIJ) continuará control en reumatología adulto. La clasificación de la Liga Internacional de Asociaciones de Reumatología (ILAR) vigente, actualmente en revisión, difiere de la clasificación de las artritis inflamatorias del adulto. Se ha reportado cambios de categoría en 10,8% de los pacientes durante el seguimiento. Objetivo: Analizar los pacientes con AIJ seguidos al menos 7 años para objetivar cambios de diagnós tico en la transición, e identificar factores de mal pronóstico funcional. Pacientes y Método: Estudio retrospectivo en base a registros clínicos. Se incluyó a la totalidad de los pacientes con AIJ controla dos en policlínico pediátrico del Hospital de Puerto Montt entre el año 2005 y 2017, que cumplieron siete o más años de seguimiento. Se realizó análisis descriptivo en base a variables clínicas: categoría diagnóstica, tiempo de evolución al diagnóstico, actividad clínica y serológica, y tiempo de evolución al inicio de la terapia farmacológica. Resultados: Se evaluaron 18 pacientes, 3 Oligo-articular (OA) persistente, 1 OA extendida, 4 Poli-articular (PA) factor reumatoide (FR) negativo, 4 PA FR positivo, 5 Sistémicas, 1 Psoriática, todos con seguimiento mayor a 7 años. Once de 18 niños fueron transfe ridos a adultos. Tres de 11 cambiaron de diagnóstico a Artritis Reumatoide (AR) más otra enferme dad autoinmune: Síndrome de Sjögren + Lupus eritematoso sistémico, Púrpura trombocitopénico inmune, Enfermedad autoinmune no clasificada y cinco de 11 niños de categoría ILAR: OA a Artritis reumatoide juvenil, OA extendida a PA FR negativo, 3 Sistémicas a PA FR negativo. Edad de inicio, formas poli-articulares, retrasos en diagnóstico y comienzo de terapia se asociaron a secuelas e infla mación persistente. Conclusiones: Ocho de once pacientes transferidos cambiaron denominación diagnóstica y/o presentaron otras enfermedades autoinmunes. Algunos factores de mal pronóstico deben mejorar.

Abstract: Introduction: At least 50% of pediatric patients with Juvenile Idiopathic Arthritis (JIA) will require continued fo llow-up in adult rheumatology. The present International League of Associations for Rheumatology (ILAR) classification, currently under revision, differs from its classification of inflammatory arthritis in adults. Category changes have been reported in 10.8% of patients during follow-up. Objective: To analyze JIA patients in follow-up for at least 7 years to detect diagnosis changes during transition to adult care, identifying factors of poor functional prognosis. Patients and Method: Retrospective study based on medical records of JIA patients seen at the pediatric polyclinic of the Puerto Montt Hospital between 2005 and 2017, who were monitored for at least 7 years. Descriptive analysis was performed according to clinical variables: diagnostic category, evolution before diagnosis, clinical and serological activity, and evolution before starting drug therapy. Results: We evaluated 18 pa tients, corresponding to 3 patients with persistent oligoarticular arthritis (OA), 1 with extended OA, 4 with polyarticular arthritis (PA) rheumatoid factor (RF) negative, 4 with PA RF positive, 5 with syste mic JIA, and 1 with psoriatic arthritis, all have had follow-up more than 7 years. 11 out of 18 patients transitioned to adult care. Three out of 11 patients changed diagnosis to Rheumatoid Arthritis (RA) plus another autoimmune disease such as Sjögren's Syndrome + Systemic Lupus Erythematosus, Immune thrombocytopenia, or unclassified autoimmune disease, and 5 out of 11 children changed ILAR category from OA to Juvenile Rheumatoid Arthritis, extended OA to PA RF negative, and 3 from Systemic arthritis to PA RF negative. Age of onset, polyarticular forms, delay in diagnosis, and the start of therapy were associated with sequelae and persistent inflammation. Conclusions: Eight of the eleven JIA patients who transitioned to adult care changed their diagnosis or presented other autoimmune diseases. Some factors of poor prognosis must improve.

Dry eye in systemic lupus erythematosus patients / Olho seco em pacientes com lúpus eritematoso sistêmico

Abstract Objective: To study the association of dry eye with lupus disease activity and cumulative damage. To verify if epidemiological, treatment and autoantibody profile of SLE (systemic Lupus erythematosus) patients influence the presence of dry eye. Methods: We studied 70 SLE patients for the presence of dry eye by Schirmer test, disease activity by SLEDAI (SLE-Disease activity index) and cumulative damage by SLICC/ACR DI (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index). Patients were also submitted to the OSDI (Ocular Surface Disease Index) questionnaire. Epidemiological and treatment data and autoantibody profile were extracted from the charts. Results: Dry eye by Schirmer test was present in 51.4% of the sample. No association of the presence of dry eye with SLEDAI and SLICC DI were found (p = ns). Subjective symptoms of dry eye measured by OSDI showed a modest correlation with SLEDAI (Spearman rho = 0.32). Treatment profile did not influence in the presence of dry eye that was more common in older patients (p < 0.0001). Anti dsDNA had a negative association with the presence of positive Schirmer test (p = 0.0008). Conclusions: Dry eye detected by Schirmer test in SLE patients has no association with disease activity nor cumulative damage. Anti dsDNA seems to have a protective effect in this context.

Resumo Objetivos: Estudar a associação do olho seco com a atividade do lúpus eritematoso sistêmico (LES) e seus danos cumulativos. Verificar se o perfil epidemiológico, de tratamento e de auto anticorpos de pacientes com LES influencia a presença de olho seco. Métodos: Foram estudados 70 pacientes com LES para a presença de olho seco pelo teste de Schirmer, atividade da doença por SLEDAI (SLE Disease Activity Index) e dano cumulativo por SLICC/ACR DI (Clínicas Colaborativas Internacionais de Lúpus Eritematoso Sistêmico/American College of Rheumatology Damage Index). Os pacientes também foram submetidos ao questionário OSDI (índice de doenças da superfície ocular). Os dados epidemiológicos e de tratamento e o perfil de auto anticorpos foram extraídos dos prontuários. Resultados: Olho seco pelo teste de Schirmer esteve presente em 51,4% da amostra. Nenhuma associação da presença de olho seco com SLEDAI e SLICC/ACR DI foi encontrada (p = ns). Os sintomas subjetivos do olho seco medidos por OSDI mostraram uma correlação modesta com SLEDAI (Rho de Spearman = 0,32) . O perfil do tratamento não influenciou na presença de olho seco que era mais comum em uns pacientes mais idosos (p < 0, 1). Anti dsDNA teve uma associação negativa com a presença de teste positivo de Schirmer (p = 0, 8). Conclusões: Olho seco detectado pelo teste de Schirmer em pacientes com LES não tem associação com atividade da doença nem dano cumulativo. Anti dsDNA parece ter um efeito protetor neste contexto.

Lupus eritematoso sistémico: impacto educativo en la calidad de vida de los pacientes / Systemic lupus erythematosus: educational impact on the quality life of patients

Objetivo: analizar el impacto de un programa educativo y su efecto en la calidad de vida de los pacientes con lupus eritematoso sistémico adscritos a la unidad de reumatología del Hospital Dr. Miguel Pérez Carreño durante el 2016. Métodos: se realizó un estudio experimental, donde se comparó dos grupos: 1 grupo al cual se intervino educativamente sobre Lupus, y 1 grupo donde se intervino educativamente sobre otros temas relacionados con la salud, todos, pacientes adscritos a la unidad de reumatología del Hospital Pérez Carreño. Resultados: se incluyeron 50 pacientes en el estudio, 25 en cada grupo. Se encontró un bajo nivel de conocimiento sobre su patología en ambos grupos (puntuación media: 9,4 experimental vs 11,1 control). La calidad de vida relacionada con la salud tanto física como mental no fue favorable pre intervención educativa en los dos grupos. La adherencia al tratamiento mejoró luego de la intervención educativa (p<0,05). Conclusión: Los resultados de la investigación indican que el impacto del programa educativo fue favorable al mejorar de forma significativa el conocimiento de la enfermedad, la calidad de vida física y mental, lograr una mejor adherencia al tratamiento indicado y disminuyendo a su vez el nivel de actividad del LES(AU)

Objective: To analyze the impact of an educational program in patients with systemic lupus erythematosus and its effect on the quality of life attached to the unit rheumatology Dr. Miguel Perez Carreño Hospital during 2016. Methods: An experimental study where two groups were compared: 1 group which was intervened educationally about Lupus and 1 group which was intervened educationally about other health-related issues. All patients were chosen from the unit of rheumatology at the Hospital Dr Miguel Perez Carreño. Results: 50 patients were included in the study, 25 in each group. It was demonstrated in both groups no knowledge about their condition (points mean: experimental: 9.4 vs control: 11.1). the quality of life related to health in both groups both physical and mental was not favorable pre educational intervention. Adherence to treatment improved ther the educational intervention (p <0.05). Conclusion: the results of the research indicate that the impact of the educational program was favorable by significantly improving the knowledge of the disease, the quality of physical and mental life, achieving a better adherence to the indicated treatment and decreasing in turn the level of activity of the LES(AU)

The effect of therapies on the quality of life of patients with systemic lupus erythematosus: a meta-analysis of randomized trials

Abstract Introduction Systemic lupus erythematosus (SLE) is a multi-systemic, chronic inflammatory disease of autoimmune nature, which can impair performance in daily life activities, causing to a compromised quality of life. Thus, the aim of this study was to evaluate the effect of therapies, such as physical activity, cognitive behavioral therapy, pharmacological treatment and phytotherapy in the quality of life of patients with systemic lupus erythematosus. Materials and methods A systematic review with a meta-analysis of randomized clinical trials was conducted by searching the PubMed database, including studies comparing patients who participated in cognitive therapy, physical activity, pharmacological treatment or phytotherapeutic treatment. Results Of the seven studies included in this meta-analysis, a significant difference was observed in the quality of life of patients with lupus who participated in the intervention groups compared to the control groups (− 10.27 95% CI: − 15, 77 at − 4.77, p = 0.0003, I2 = 0%). Conclusion Interventions improve the Quality of life of patients with SLE. However, the methodological quality of the included articles and the sizes of the samples for being small propose that new randomized clinical trials be performed.(AU)

Systemic lupus erythematosus and pregnancy: A single-center observational study of 69 pregnancies / Lúpus eritematoso sistêmico e gravidez: estudo observacional em um único centro com 69 gestações

Abstract Objective To evaluate the effects of pregnancy in systemic lupus erythematosus (SLE) patients. Methods The present article is a retrospective cohort study. Datawere collected from medical records of pregnant women with SLE from January 2002 to December 2012 at Universidade Estadual de Campinas, in the city of Campinas, state of São Paulo, Brazil. Systemic lupus erythematosus and disease activity were defined according to the American College of Rheumatology and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) criteria respectively. The means, standard deviations (SDs), percentages and correlations were performed using the SAS software, version 9.4 (SAS Institute Inc., Cary, NC, US). Results We obtained data from 69 pregnancies in 58 women. During pregnancy, a new flare was observed in 39.2% (n = 27). The manifestations were most common in patients with prior kidney disease, and mainly occurred during the third quarter and the puerperium. Renal activity occurred in 24.6% (n = 17), and serious activity, in 16% (n = 11). Of all deliveries, 75% (n = 48) were by cesarean section. Twomaternal deaths occurred (3%). Preterm birth was themain complication in the newborns. The abortion rate was 8.7%. Severe SLEDAI during pregnancy was associated with prematurity (100%) and perinatal death (54%). Conclusion Thematernal-fetal outcome is worse in SLE when thewomen experience a flare during pregnancy. The best maternal-fetal outcomes occur when the disease is in remission for at least 6 months before the pregnancy.

Resumo Objetivo Avaliar os efeitos da gravidez em pacientes com lúpus eritematoso sistêmico (LES). Métodos Estudo de coorte retrospectivo. Os dados foram coletados de prontuários de mulheres com LES que engravidaram de janeiro de 2002 a dezembro de 2012 na Universidade Estadual de Campinas, São Paulo, Brasil. Lúpus eritematoso sistêmico e atividade da doença foram definidos segundo o American College of Rheumatology e os critérios doÍndice deAtividadedaDoença de Lúpus Eritematoso (SLEDAI, nasigla eminglês), respectivamente. As médias, os desvios-padrão (DP), as porcentagens e as correlações foram realizados utilizando o software SAS, versão 9.4 (SAS Institute Inc., Cary, NC, US). Resultados Obtivemos dados de 69 gestações em58mulheres. Durante a gravidez, a reatividade da doença foi observada em 39.2% (n = 27). As manifestações mais comuns foram em pacientes com doença renal prévia, e ocorreram principalmente no terceiro trimestre e no puerpério. Atividade renal ocorreu em 24,6% (n = 17), e atividade grave, em 16% (n = 11). De todos os partos, 75% (n = 48) foram por cesariana. Dois óbitos maternos ocorreram (3%). A prematuridade foi a principal complicação nos recém-nascidos. A taxa de aborto foi de 8,7%. O índice SLEDAI grave durante a gestação foi associado à prematuridade (100%) e à morte perinatal (54%). Conclusão O resultado materno-fetal é pior no LES quando as mulheres sofrem crise de reativação durante a gravidez. Os melhores desfechos materno-fetais ocorrem quando a doença está em remissão por pelo menos 6 meses anteriores à gestação.

A critical review on obstetric follow-up of women affected by systemic lupus erythematosus / Uma revisão crítica sobre o acompanhamento obstétrico de mulheres com lúpus eritematoso sistêmico

Abstract Objective To review the existing recommendations on the prenatal care of women with systemic lupus erythematosus (SLE), based on currently available scientific evidence. Methods An integrative review was performed by two independent researchers, based on the literature available in the MEDLINE (via PubMed), EMBASE and The Cochrane Library databases, using the medical subject headings (MeSH) terms "systemic lupus erythematosus" AND "high-risk pregnancy" OR "prenatal care." Studies published in English between 2007 and 2017 were included; experimental studies and case reports were excluded. In cases of disagreement regarding the inclusion of studies, a third senior researcher was consulted. Forty titles were initially identified; four duplicates were excluded. After reading the abstracts, 7 were further excluded and 29 were selected for a full-text evaluation. Results Systemic lupus erythematosus flares, preeclampsia, gestation loss, preterm birth, fetal growth restriction and neonatal lupus syndromes (mainly congenital heartblock) were the major complications described. The multidisciplinary team should adopt a specific monitoring, with particular therapeutic protocols. There are safe and effective drug options that should be prescribed for a good control of SLE activity. Conclusion Pregnant women with SLE present an increased risk for maternal complications, pregnancy loss and other adverse outcomes. The disease activity may worsen and, thereby, increase the risk of other maternal-fetal complications. Thus, maintaining an adequate control of disease activity and treating flares quickly should be a central goal during prenatal care.

Resumo Objetivo Revisar as recomendações existentes sobre o cuidado pré-natal às mulheres comlúpus eritematoso sistêmico (LES), combase emevidências científicas atualmente disponíveis. Métodos Revisão integrativa realizada por dois pesquisadores independentes, com base na literatura disponível nos bancos de dados MEDLINE (via PubMed), EMBASE e The Cochrane Library, usando os cabeçalhos de assuntos médicos, ou termos MeSH, "systemic lupus erythematosus" E "high-risk pregnancy" OU "prenatal care." Estudos publicados em inglês entre 2007 e 2017 foram incluídos; estudos experimentais e relatos de caso foram excluídos. Em caso de desacordo, umterceiro pesquisador sênior foi consultado. Quarenta títulos foram inicialmente identificados; quatro duplicatas foram excluídas. Após leitura dos resumos, mais 7 artigos foramexcluídos e 29 foram selecionados para uma avaliação de texto completo. Resultados Surtos de LES, pré-eclâmpsia, perda de gestação, parto prematuro, restrição de crescimento fetal e síndromes de lúpus neonatal foram as principais complicações descritas. A equipe multidisciplinar deve adotar um monitoramento específico, com protocolos terapêuticos apropriados. Há drogas seguras e eficazes que devem ser prescritas para um bom controle do LES. Conclusão Gestantes com LES apresentam risco aumentado de complicações maternas, perda de gravidez e outros desfechos adversos. A atividade da doença pode piorar e, assim, aumentar o risco de outras complicações. Assim, manter um controle adequado da atividade da doença e tratar rapidamente os surtos deve ser um objetivo central durante o pré-natal.

Retinopatia vaso-oclusiva por lúpus eritematoso sistêmico associada à síndrome do anticorpo antifosfolipídeo / Vaso-occlusive retinopathy by systemic lupus erythematosus associated with the antiphospholipid antibody syndrome

Resumo Relatar um caso de paciente com Retinopatia vaso-oclusiva por Lúpus Eritematoso Sistêmico (LES) associado à Síndrome do Anticorpo Antifosfolipídeo (SAF), que se iniciou com um quadro de anemia hemolítica autoimune acompanhado por baixa visual súbita monocular. Poucos casos foram descritos na literatura nacional e mundial em que o LES se manifeste primeiramente com alterações oculares. O screening dos Anticorpos antifosfolípideos (APAs) é de suma importância para pacientes com retinopatia lúpica para que seja instituída a terapia imediata com anticoagulantes como forma de prevenir a trombose vascular, o que piora o prognóstico visual.

Abstract To report the case of a patient with vaso-occlusive retinopathy due to systemic lupus erythematosus (SLE) associated with antiphospholipid antibody syndrome (APAS), which started with signs and symptoms of autoimune hemolytic anemia accompanied by sudden monocular visual loss. Few cases of SLE manifestation primarily involving ocular changes have been reported in the Brazilian and international literature. Screening for antiphospholipid antibodies is of the greatest importance for patients with lupus retinopathy, so that immediate therapy with anticoagulants may be instituted in order to prevent vascular thrombosis, which worsens the visual prognosis.

Lupus eritematoso sistémico en una zona austral de Chile / Systemic lupus erythematosus in a southern zone of Chile

Introducción. El lupus eritematoso sistémico (LES) se caracteriza por un amplio espec-tro de manifestaciones clínicas y serológicas. La última serie de enfermos chilenos fue publicada el año 1994 y contempló un total de 218 pacientes. Objetivo. Contribuir con nuevos datos epidemiológicos en una cohorte de 67 enfer-mos con LES en una zona austral de nuestro país.Pacientes y métodos. De forma retrospectiva se revisaron 100 fichas de pacientes con diagnóstico de LES realizado por médico reumatólogo del Hospital de Puerto Montt (HPM), identificados a través de registros obtenidos a contar de julio de 2013 cuando esta patología se incluyó dentro de las Garantías Explícitas en Salud (GES). 67 cumplían al menos cuatro criterios Systemic Lupus International Collaborating Clinics (SLICC) 2012 y los datos obtenidos fueron sometidos a análisis estadístico utilizando el programa SPSS versión 15.0. Resultados: 63 (94%) son mujeres. La edad promedio al momento del diagnóstico fue 38,1 años y el tiempo transcurrido desde el inicio de los síntomas fue 12,3 me-ses. Las manifestaciones clínicas más frecuentes fueron sinovitis (65.7%), alopecia (47.8%), compromiso renal (43.3%) y alteraciones cutáneas (38.8%). Sesenta (90%) tenían ANA positivo, 34 (50.7%) anti-DNA positivo y 45 (67.2%) hipocomplementemia. Se identificaron 29 (43.3%) pacientes con compromiso clínico y/o histológico renal, de los cuales a 13 se les realizó biopsia renal, correspondiente en el 69% de los casos a nefritis lúpica membrano-proliferativa.Conclusiones. Nuestra cohorte no difiere sustantivamente de otras, salvo por la baja prevalencia de manifestaciones cutáneas. Se detectó la falta de acceso a biopsia renal y exámenes serológicos autoinmunes dado principalmente por falta de profesionales idóneos en nuestra zona que permitan el acceso universal a estos procedimientos.Este estudio permite proporcionar información actualizada de pacientes con LES en una zona austral de Chile.

Introduction. Systemic lupus erythematosus (SLE) is characterized by a broad spec-trum of clinical and serological manifestations. The last series of chilean patients was published in 1994 and included a total of 218 patients.Objective. To contribute with new epidemiological data in a cohort of 67 patients with SLE in a Southern area of our country.Patients and methods. In a retrospective way, 100 records of patients diagnosed with SLE were reviewed by a rheumatologist at the Hospital de Puerto Montt (HPM), identified through records obtained as of July 2013 when this pathology was included in the Explicit Health Guarantees. (GES) 67 met at least four criteria Systemic Lupus International Collaborating Clinics (SLICC) 2012 and the data obtained were subjected to statistical analysis using the SPSS program version 15.0.Results: 63 (94%) are women. The average age at diagnosis was 38.1 years and the time elapsed since the onset of symptoms was 12.3 months. The most frequent clinical manifestations were synovitis (65.7%), alopecia (47.8%), renal involvement (43.3%) and skin alterations (38.8%). Sixty (90%) had positive ANA, 34 (50.7%) positive anti-DNA and 45 (67.2%) hypocomplementemia. We identified 29 (43.3%) patients with clinical and / or renal histological involvement, of whom 13 were under-going a renal biopsy corresponding to 69% of the cases of membranous proliferative lupus nephritis.Conclusions. Our cohort does not differ substantively from others, except for the low prevalence of cutaneous manifestations.The lack of access to renal biopsy and self-immune serological tests was detected, mainly due to the lack of qualified professionals in our area that allow universal access to these procedures.This study allows us to provide updated information on patients with SLE in a Southern zone of Chile.

Efficacy of mesenchymal stem cells on systemic lupus erythematosus:a meta-analysis / 北京大学学报(医学版)

OBJECTIVE@#Systemic lupus erythematosus (SLE) is an autoimmune disease with multi-organ involvement and several typical autoantibodies. Mesenchymal stem cells (MSC) are multipotent stem cells with low immunogenicity that can differentiate into various kinds of cells, such as bone, cartilage, fat and skin tissue. MSC have immunomodulatory and reparative properties through interactions with immune cells. MSC have been used in the treatment of refractory SLE and lupus nephritis patients for more than ten years. Most clinical studies were self-controlled studies and only a few were randomized controlled trials. The objective of this study was to use meta-analysis method to evaluate the efficacy and safety of MSC treatment in SLE patients.@*METHODS@#The PubMed, Cochrane Library, Wanfang and VIP databases were searched for published randomized controlled trials and self-controlled studies before June 1, 2018. The search terms included the Chinese and English versions of mesenchymal stem cells, Mesenchymal Stromal Cells [Mesh], systemic lupus erythematosus, lupus, Lupus Erythematosus, Systemic [Mesh]. Two authors independently screened the literatures, assessed the quality of the studies and collected data according to the inclusion and exclusion criteria. The endpoints were the SLE disease activity index, 24 h urine protein and complement C3. Meta-analysis was performed with the Revman 5.3 software according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standard.@*RESULTS@#Eight studies involving 213 patients were included and three of the studies were randomized controlled trials with 66 patients involved. The MSC group showed that the SLE disease activity index decreased significantly [standard mean difference (SMD)=-1.76, 95% confidence interval (CI): -2.00 to -1.51, P<0.001), the 24 h urine protein decreased significantly (SMD=-1.74, 95%CI: -2.46 to -1.03, P<0.001), as well as the complement C3 increased significantly (SMD=1.28, 95%CI: 0.93 to 1.62, P<0.001). Four studies reported adverse events including fever, diarrhea and headache during the infusion.@*CONCLUSION@#Current evidences showed that MSC could improve the disease activity, proteinuria and hypocomplementemia in SLE patients. Large scale and high-quality randomized controlled trials are required to validate the efficacy and safety of MSC treatment in SLE patients.

Atuação fisioterapêutica em um indivíduo com lúpus eritematoso sistêmico associado à artrite reumatoide e à fibromialgia / Physiotherapy performance in an individual with systemic lupus erythematosus associated with rheumatoid arthritis and fibromyalgia

INTRODUÇÃO: As doenças reumáticas podem manifestar-se de forma isolada ou concomitante, como, por exemplo, o lúpus eritematoso sistêmico, que pode associar-se à artrite reumatoide e à fibromialgia, comprometendo a funcionalidade e a qualidade de vida. O objetivo deste estudo foi verificar os efeitos da fisioterapia na dor, na força de preensão palmar, no equilíbrio postural, no nível de incapacidade e na qualidade de vida de um indivíduo com lúpus eritematoso sistêmico associado à artrite reumatoide e à fibromialgia. RELATO DE CASO: Mulher, 56 anos, diagnosticada com lúpus eritematoso sistêmico, artrite reumatoide e fibromialgia há 14 anos. Sua queixa principal era dor generalizada e intensa em diversos pontos do corpo. Foram avaliados a dor, a força de preensão palmar, o equilíbrio postural, o nível de incapacidade e a qualidade de vida. O indivíduo realizou 30 sessões de fisioterapia (cinesioterapia e hidrocinesioterapia), duas vezes por semana, entre os meses de agosto e novembro de 2015, na Clínica de Fisioterapia da Universidade de Passo Fundo. Após a intervenção, houve diminuição da dor em 5 pontos e da incapacidade em 30%; aumento da força de preensão palmar em 2,2 kgf (mão direita) e 1,2 kgf (mão esquerda); e do equilíbrio postural em 6 pontos. Como consequência, a qualidade de vida do indivíduo melhorou. CONCLUSÃO: A fisioterapia demonstrou-se eficaz na diminuição da dor e da incapacidade, no aumento da força de preensão palmar bilateral e do equilíbrio postural e na melhora da qualidade de vida em um indivíduo com lúpus eritematoso sistêmico associado à artrite reumatoide e à fibromialgia.

INTRODUCTION: Rheumatic diseases may occur singularly or in concomitant way, such as systemic lupus erythematosus, that may be associated with rheumatoid arthritis and fibromyalgia, compromising the functionality and quality of life. The aim of this study was to investigate the effects of physiotherapy on pain, handgrip strength, postural balance, incapacity level and quality of life in an individual with systemic lupus erythematosus associated with rheumatoid arthritis and fibromyalgia. CASE REPORT: Woman, 56 years old, diagnosed with systemic lupus erythematosus, rheumatoid arthritis and fibromyalgia for 14 years. Her main complaint was widespread and intense pain in several points of the body. We evaluated pain, handgrip strength, postural balance, incapacity level and quality of life. The individual performed 30 sessions of physiotherapy (kinesiotherapy and hydrokinesiotherapy), twice a week, between August and November 2015, at Physiotherapy Clinic of the University of Passo Fundo. After the intervention, there was reduction of pain by 5 points and incapacity by 30%, increased handgrip strength in 2,2 kgf (right hand) and 1,2 kgf (left hand) and postural balance in 6 points. As a consequence, the individual's quality of life improved. CONCLUSION: The physiotherapy proved to be effective in reducing pain and disability, increasing bilateral handgrip strength and postural balance and improving the quality of life of an individual with systemic lupus erythematosus associated with rheumatoid arthritis and fibromyalgia.

Tratamiento psicológico en pacientes lupus eritematoso sistémico: una revisión sistemática / Psychological treatment in patients with systemic lupus erythematosus: a systematic review

El propósito de esta revisión sistemática fue explorar las intervenciones psicológicas aplicadas a pacientes con lupus eritematoso sistémico (LES) y analizar su utilidad para promover una adaptación favorable en el área psicológica, social y física. Se realizó una exhaustiva búsqueda en las bases de datos Scopus, Medline, PsycINFO y Cochrane Library y se seleccionaron investigaciones empíricas publicadas entre 2005 y 2016. Los estudios reflejaron diferentes opciones terapéuticas: terapia cognitivo-conductual, expresión emocional escrita, entrenamiento en atención plena y una combinación de psicoeducación y psicoterapia en grupo. Los resultados muestran que estas intervenciones pueden repercutir positivamente en la calidad de vida, la ansiedad, la depresión, el estrés, la salud mental, la imagen corporal, el manejo de la enfermedad, las relaciones interpersonales, la fatiga, y el dolor. Sin embargo, los estudios presentan notables limitaciones metodológicas que impiden extraer conclusiones definitivas. Por ello, se considera imprescindible la realización de ensayos controlados aleatorizados de mayor calidad metodológica que permitan cuantificar la eficacia de las intervenciones psicológicas en LES y establecer la superioridad de un tratamiento frente a otro.

The purpose of this systematic review was to explore psychological interventions applied to patients with systemic lupus erythematosus (SLE) and analyze their usefulness in promoting a favorable adaptation in the psychological, social and physical area. A comprehensive search bases Scopus, Medline, PsycINFO and Cochrane Library data was performed and empirical research published between 2005 and 2016 were selected studies reflected different therapeutic options: cognitive behavioral therapy, emotional writing, and mindfulness training a combination of group psychoeducation and psychotherapy. The results show that these interventions can positively impact the quality of life, anxiety, depression, stress, mental health, body image, disease management, interpersonal relationships, fatigue, and pain. However, studies have significant methodological limitations that prevent definitive conclusions. Therefore, it is considered essential to perform randomized controlled trials of higher methodological quality to quantify the effectiveness of psychological interventions in sle and establish the superiority of one treatment over another.

Influence of medical care in the quality of life (WHOQOL-100) of women living with systemic lupus erythematosus / Influência da atenção médica na qualidade de vida (WHOQOL-100) de mulheres com lúpus eritematoso sistêmico

ABSTRACT Lupus requires careful treatment by medical specialists. This study aimed to evaluate the quality of life of patients living with Systemic Lupus Erythematosus (SLE). The method approached women with a confirmed diagnosis of SLE under medical supervision in a University hospital outpatient clinic or in a private clinic. We used an instrument containing relevant information of the patient and also the Portuguese version of the World Health Organization Quality of Life Group (WHOQOL-100) questionnaire. The study population consisted of 39 women, married in their majority (56.4%); prevalently aged 37–60 years old; prevalence of diagnosis time over five years (66.7%); and in use of less than 5 medications (69.2%). 92.3% had the disease inactive with higher means in mobility (p = 0.0463) and quality of life (p = 0.0199) facets; on the other hand, the physical safety and security facet (p = 0.0093) showed higher mean for people with active disease. Health and social care analysis showed availability and quality (p = 0.0434), even when with other associated diseases (33.3%); and the highest means were Dependence on medicinal substances and medical aids (p = 0.0143). The negative results for associated diseases were higher in sexual activity (p = 0.0431) and transportation (p = 0.0319) facets. In conclusion: if women living with SLE receive continuous medical attention, they will enjoy good quality of life, while minimizing the complications inherent in this condition.

RESUMO O lúpus exige tratamento cuidadoso por médicos especialistas. Este estudo teve como objetivo avaliar a qualidade de vida de pacientes com lúpus eritematoso sistêmico (LES). O método abordou mulheres com diagnóstico confirmado de LES sob acompanhamento médico em ambulatório de hospital escola ou particular. Usou-se instrumento que continha informações relevantes do paciente e a versão em português do instrumento de pesquisa World Health Organization Quality of Life Group (WHOQOL-100). A população estudada perfez 39 mulheres, na sua maioria casadas (56,4%); com idade prevalente de 37 a 60 anos; tempo de diagnóstico prevalente maior de cinco anos (66,7%); uso de menos de cinco medicamentos (69,2%). Obteve-se que 92,3% estavam com a doença controlada com médias maiores nas facetas mobilidade (p = 0,0463) e qualidade de vida (p = 0,0199). Já a faceta segurança física e proteção (p = 0,0093) apresentou média maior para pessoas com a doença em estado ativado. As análises de cuidados de saúde e sociais apresentaram disponibilidade e qualidade (p = 0,0434) mesmo com outras patologias associadas (33,3%) e as maiores médias foram dependência de medicação ou de tratamentos (p = 0,0143). Os resultados negativos para doença associada foram maiores nas facetas atividade sexual (p = 0,0431) e transporte (p = 0,0319). Conclui-se que se a mulher com LES receber atenção médica de forma continua apresentará qualidade de vida que minimizará as complicações inerentes a essa patologia.

Development of a smartphone app for adolescents with lupus: a collaborative meeting-based methodology inclusive of a wide range of stakeholders / Desarrollo de una aplicación para teléfonos móviles inteligentes dirigida a los adolescentes con lupus: un método colaborativo basado en reuniones con una amplia gama de interesados directos

Traditional challenges of creating a medical app include hearing the voices of various stakeholders as a collective rather than in a consultative process that is sequential. This report describes the development of a mobile (smartphone) app for adolescents with lupus as well as the process that was used to overcome the challenge described above. The development of the smartphone app addressed optimal ways to incorporate information about 1) lupus, including the effects of both the disease and the medications used to treat it; 2) how life choices can affect lupus patients' condition; and 3) ways to increase self-management and communication. The collaborative concept-generating and requirements-gathering methodology was used during a two-day workshop with a range of stakeholders (ages 16 - 59 years) that focused on leveraging user-centered design methods to generate guidance to mobile app developers. The app development process conducted during the workshop included the following steps: 1) recruiting a goal-focused collaborative group, 2) defining app objectives, 3) evaluating potential needs of users, 4) brainstorming app features and use-case modeling, 5) reviewing existing app features and prototypes, 6) refining functionalities, 7) writing user narratives, 8) visualizing navigation and feature design, and 9) identifying content. The use of creative devices such as drawing interfaces fostered fun, engagement, and sustained energy, and the use of a brainstorming technique leveraged methods that ensured an inclusive process so that even participants who were shy, quiet, or easily intimidated by "professionals" felt confident to contribute. In addition to a name change for the app, project outcomes included the selection of the following app features: symptom tracking; appointment and medication reminders; a social media component; a medical summary; easy navigation; informational content; gamification; and personalization (options for customization).

Uno de los desafíos tradicionales durante el desarrollo de una aplicación médica es considerar las opiniones de los diversos interesados directos como colectivo, en lugar de emplear un proceso de consulta de tipo secuencial. En este informe se describe el desarrollo de una aplicación para teléfonos móviles inteligentes dirigida a adolescentes con lupus, así como el procedimiento empleado para superar este tipo de dificultades. En el desarrollo de esta aplicación se buscó la mejor manera de incorporar información acerca de: 1) el lupus, incluidos los efectos tanto de la enfermedad como de los medicamentos utilizados para su tratamiento; 2) cómo las opciones de vida pueden afectar a la situación de los pacientes con lupus; y 3) los procedimientos para aumentar el autotratamiento y la comunicación. En un taller de dos días, en el que participaron diversos interesados directos (de 16 a 59 años de edad), se empleó una metodología colaborativa de generación de conceptos y recopilación de requisitos con el propósito de aprovechar los métodos de diseño centrados en el usuario para que sirvan de guía a los productores de aplicaciones para telefonía móvil. El proceso de desarrollo de la aplicación que se llevó a cabo durante el taller utilizó los siguientes pasos: 1) captar un grupo colaborativo centrado en las metas, 2) definir los objetivos de la aplicación, 3) evaluar las posibles necesidades de los usuarios, 4) hacer una lluvia de ideas sobre las características de la aplicación y elaborar modelos de casos de uso, 5) analizar las características y los prototipos de las aplicaciones existentes, 6) perfeccionar las funcionalidades, 7) redactar distintas experiencias de los usuarios, 8) visualizar el diseño de la navegación y las funcionalidades, y 9) determinar el contenido. El uso de recursos creativos como las interfaces para dibujar fomentó la diversión, la participación y la energía sostenida; y el empleo de una técnica de lluvia de ideas permitió aprovechar algunos métodos que garantizaban un proceso inclusivo, de manera que aun los participantes tímidos, callados o fácilmente intimidables por los "profesionales" se sintieran cómodos para participar. Además del cambio de nombre de la aplicación, otro resultado del proyecto fue que se seleccionaron las siguientes características de la aplicación: seguimiento de síntomas; recordatorio de citas y medicación; un componente de redes sociales; un resumen del historial médico; navegación sencilla; contenido informativo; ludificación; y personalización (opciones de adaptación individualizada).

Lupus erythematosus: considerations about clinical, cutaneous and therapeutic aspects

Systemic Lupus Erythematosus is a chronic inflammatory disease with multifactorial etiology. Although clinical manifestations are varied, the skin is an important target-organ, which contributes to the inclusion of skin lesions in 4 out of the 17 new criteria for the diagnosis of the disease, according to the Systemic Lupus International Collaborating Clinics. The cutaneous manifestations of lupus are pleomorphic. Depending on their clinical characteristics, they can be classified into Acute Cutaneous Lupus Erythematosus, Subacute Cutaneous Lupus Erythematosus, Chronic Cutaneous Lupus Erythematosus and Intermittent Cutaneous Lupus Erythematosus. Treatment is based on preventive measures, reversal of inflammation, prevention of damage to target organs and relief of adverse events due to pharmacological therapy. The most commonly used treatment options are topical, systemic and surgical treatment, as well as phototherapy. The correct handling of the cases depends on a careful evaluation of the morphology of the lesions and the patient's general status, always taking into consideration not only the benefits but also the side effects of each therapeutic proposal.

Terapia biológica en enfermedades reumatológicas / Biologic therapies in rheumatic diseases

El advenimiento del uso de terapias biológicas en Reumatología ha modificado significativamente el pronóstico de pacientes portadores de artritis reumatoide (AR), artritis juvenil (AJ), espondilitis anquilosante (EA), entre otras enfermedades. A diferencia de las terapias convencionales estos productos biológicos se dirigen a los llamados blancos terapéuticos ya sea estas una línea celular, un mediador inflamatorio o un receptor de superficie. Estos compuestos son producidos por células vivas mediante la tecnología del ADN recombinante. Estos compuestos pueden tener componentes humano y animal [quiméricos (Xi), humanizados (Zu)], o completamente humanos (H) lo cual se reconoce por las letras que se incluyen en el nombre de cada uno. En el campo de la Reumatología, el primer compuesto utilizado fue el etanercept (anti-factor de necrosis tumoral o anti-TNF) aprobado en 1998, pero otros anti-TNF han demostrado su beneficio en AR, como en EA y AJ. Los inhibidores de Interleucina (IL-1) casi no se usan en AR actualmente, pero si los inhibidores de IL-6, así como los agentes contra las células B y los agonistas de CTLA-4 (Cytotoxic T lymphocyte antigen). Existe asimismo un compuesto dirigido al BLyS (B-lymphocyte stimulator) el cual se usa en lupus eritematoso sistémico y otro dirigido al receptor activador del factor nuclear κB (RANKL, receptor activator of nuclear factor-κB ligand) que se usa en osteoporosis. Con el avance en el conocimiento de la patogenia de las enfermedades reumáticas, se vienen reconociendo otra blancos terapéuticas. En los años venideros, este campo ha de expandirse en proporciones geométricas.

The advent of biologic therapies in Rheumatology has modified significantly the prognosis of patients with rheumatoid arthritis (RA), juvenile arthritis (JA), ankylosing spondylitis (AS), among others. In contrast to the conventional therapies, these biological therapies are directed at specific targets being those a cell line, an inflammatory mediator, or a surface receptor. These compounds are produced by live cells using recombinant DNA technology. They can have human and animal components [chimerics (Xi), humanized (Zu)] or be completely human (H); this is recognized by the letters included in their names. The first compound used in Rheumatology was etanercept (antitumor necrosis factor) which was approved by the FDA in 1998; however other anti-TNF compounds have proven to be beneficial in RA as well as in AS and JA. The interleukin-1 (IL-1) inhibitors are rarely used in RA at thepresent time, but IL-6 inhibitors and agents directed against CTLA-4 (Cytotoxic T lymphocyte antigen) are. There is also a compound against BLyS (B-lymphocyte stimulator) which is used in systemic lupus erythematosus andother directed at RANKL (receptor activator of nuclear factor-κB ligand) which is used in osteoporosis. With the advances made in the understanding of the pathogenesis of the rheumatic diseases, new therapeutic targets are being recognized. In the years to come this field will expand in geometric proportions.