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Abstract Background: Only a fraction of patients with cutaneous lupus erythematosus (CLE) will eventually progress toward systemic disease (SLE). Objective: To find inflammatory biomarkers which could predict the progression of cutaneous lupus erythematosus (CLE) into systemic lupus erythematosus (SLE) using immunohistochemical (IHC) assays. Methods: Immunohistochemical markers for cytotoxic, inflammatory, and anti-inflammatory responses and morphometric methods were applied to routine paraffin sections of skin biopsies, taken from lesions of 59 patients with discoid lupus, subacute lupus, and lupus tumidus. For the diagnosis of SLE, patients were classified by both the American College of Rheumatology (ACR-82) and the Systemic Lupus International Collaborating Clinics (SLICC-12) systems. Results: Skin samples from CLE/SLE +patients presented higher expression of IL-1β (ARC-82: p = 0.024; SLICC-12: p = 0.0143) and a significantly higher number of cells marked with granzyme B and perforin (ARC: p = 0.0097; SLICC-12: p = 0.0148). Biopsies from CLE/SLE- individuals had higher expression of IL-17 (ARC-82: p = 0.0003; SLICC-12: p = 0.0351) and presented a positive correlation between the density of granzyme A+and FoxP3+ cells (ARC-82: p = 0.0257; SLICC-12: p = 0.0285) and CD8+ cells (ARC-82: p = 0.0075; SLICC-12: p = 0.0102), as well as between granulysin-positive and CD8+ cells (ARC-82: p = 0.0024; SLICC-12: p = 0.0116). Study limitations: Patients were evaluated at a specific point in their evolution and according to the presence or not of systemic disease. The authors cannot predict how many more, from each group, would have evolved towards SLE in the following years. Conclusions: In this cohort, immunohistochemical findings suggested that patients with a tendency to systemic disease will show strong reactivity for IL-1β, while those with purely cutaneous involvement will tend to express IL-17 more intensely.

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ABSTRACT Objective: to learn the meanings attributed to pregnancy loss by women with Lupus. Method: qualitative research, based on Symbolic Interactionism and Grounded Theory. Data collection took place between January and August 2022 through in-depth interviews. Data analysis went through the stages of initial and focused coding. Results: seventeen women participated. The central phenomenon “The climb to motherhood: falls and overcoming” was constructed, consisting of three categories: “Falling to the ground during the climb: the experience of pregnancy loss”; “Getting up and following the path: new attempts to conceive”; and “Remembering the journey: meanings attributed to pregnancy losses”. Final considerations: experiencing pregnancy is, analogously, like climbing a mountain, where obstacles need to be overcome to reach the summit. The experience of pregnancy loss is seen as complex, especially when there is fragility in healthcare and a lack of awareness regarding feelings of loss and grief.

Copyright of Arquivos de Ciências da Saúde da UNIPAR is the property of Associacao Paranaense de Ensino e Cultura and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors’ intention is that, by using direct and easily accessible language, aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.

Despite being a relatively rare pathology, neonatal lupus erythematosus can affect the body in different ways, with a risk of death, especially in the first three months of life. Studies show that in asymptomatic pregnant women or those with autoimmune disease with positive Ro/SSA, La/SSB and RNP antibodies, the transplacental passage of these antibodies after the 16th week can cause damage to target organs. Most of the lesions, especially the skin, gradually decrease and disappear, as these antibodies are eliminated from the circulation; however, in the heart, fibrosis can induce permanent damage. Despite the knowledge of the importance of these antibodies in the pathophysiology of neonatal lupus, it is necessary to better understand the genetic and environmental factors of the disease. Lesions caused by lupus are generally benign and self-limiting. However, atrioventricular block is an exception, causing significant morbidity and mortality. This paper seeks to focus on the investigation of neonatal lupus erythematosus, on understanding the systemic manifestations of the disease, in order to understand the involvement of fetal organs.

Abstract Objective: To determine pregnancy outcomes in women with systemic lupus erythematosus (SLE) who were treated with hydroxychloroquine in a tertiary center. Methods: A retrospective study involving pregnant women with SLE who had antenatal follow-up and delivery in between 1 January 2007 and 1 January 2017. All participants were retrospectively enrolled and categorized into two groups based on hydroxychloroquine treatment during pregnancy. Results: There were 82 pregnancies included with 47 (57.3%) in the hydroxychloroquine group and 35 (42.7%) in the non-hydroxychloroquine group. Amongst hydroxychloroquine users, there were significantly more pregnancies with musculoskeletal involvement (p = 0.03), heavier mean neonatal birthweight (p = 0.02), and prolonged duration of pregnancy (p = 0.001). In non-hydroxychloroquine patients, there were significantly more recurrent miscarriages (p = 0.003), incidence of hypertension (p = 0.01) and gestational diabetes mellitus (p = 0.01) and concurrent medical illness (p = 0.005). Hydroxychloroquine use during pregnancy was protective against hypertension (p = 0.001), and the gestational age at delivery had significant effect on the neonatal birthweight (p = 0.001). However, duration of the disease had a significant negative effect on the neonatal birthweight (p = 0.016). Conclusion: Hydroxychloroquine enhanced better neonatal outcomes and reduced adverse pregnancy outcomes and antenatal complications such as hypertension and diabetes.

Systemic lupus erythematosus may present ophthalmological involvement, usually benign, and the most common changes are dry eye syndrome and cataract. In patients with stable disease, ophthalmologic damage appears to be related to long-term systemic treatment, emphasizing the importance of routine complete ophthalmologic examination. However, in full-blown disease, especially when there is renal involvement, early treatment should start with systemic steroid therapy and supportive measures, to avoid major repercussions, such as hypertensive crises that may lead to death. [ABSTRACT FROM AUTHOR]

Objective: to clarify the relationship of glucocorticoid-induced osteonecrosis (ON) in patients with systemic lupus erythematosus (SLE) and to verify if there is a dose x duration relationship, using the systematic review method. Methods: A systematic literature search was performed on PubMed, LILACS, SciELO and Cochrane Library. This search prioritized studies published between the years 2009-2019, which involved the use of glucocorticoids as a form of treatment for SLE and which had the presence of ON as an outcome. Results: From 1,051 articles previously identified in the databases, 6 studies were selected that met the criteria established for this systematic review. The articles indicated a great tendency for the presence of NO in patients using corticosteroids, demonstrating that higher doses tend to increase the chance of this manifestation. The age of earlier diagnosis of SLE showed a greater relationship with the onset of ON, manifesting mainly in the hip joint. Conclusion: Although most studies point to the relationship of corticosteroid therapy with regard to dose and time with the onset of ON, a greater number of studies need to be evaluated, since some of our findings demonstrate this manifestation with doses equivalent to 7.5mg/day, while another found the possibility of this unfolding being typical of the course of the disease. [ABSTRACT FROM AUTHOR]

Copyright of Revista Nutrición Clínica y Dietética Hospitalaria is the property of Sociedad Espanola de Dietetica y Ciencias de la Alimentacion and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Lupus nephritis (NL) is one of the most severe consequences that affects patients with systemic lupus erythematosus (SLE). One of the diagnostic criteria for LN is glomerulonephritis. LN it divides itself into classes that vary according to kidney injury. Renal biopsy in the evaluation of impairment of the renal system in patients with SLE has become necessary to define the degree of impairment and the class of LN in which the patient is. It is a more common disease in women in the reproductive phase, being among nine women for each man affected by the disease, and with the variant prevalence of 14 to 50/100,000 inhabitants. This work is a descriptive and exploratory bibliographic review based on scientific articles found between 1988 and 2020. The present study consists in the early identification of the impairment of the renal system in patients with SLE, in order to try to prevent and/or delay the advance of the disease to its final stage, which is renal failure. [ABSTRACT FROM AUTHOR]

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.

It's reported the case of a 37-year-old female patient with diagnosed Systemic Lupus Erythematosus (ACR / EULAR criteria, 2019: FAN 1: 160 homogeneous nucleolar and nuclear fine speckled mixed; autoimmune hemolytic anemia; anti-DNAds positive; anti-beta-2-glycoprotein-1 IgG), in moderate disease activity (SLEDAI-2K = 8), who started treatment with hydroxychloroquine 400 mg / day. Approximately three weeks after starting the medication, he developed diffuse maculopapular erythema, associated with pruritus that evolved with secondary infection and need for hospitalization. Skin biopsy was performed, which revealed changes compatible with pharmacoderma. After suspension of hydroxychloroquine and treatment with oxacillin, there was complete resolution of the condition. This work highlights the importance of following up patients with recent diagnoses and introducing new medications, even though it is a widely used safe drug, emphasizing that adverse reaction monitoring is a valuable element in the monitoring and appropriate treatment. [ABSTRACT FROM AUTHOR]

These are cross-cutting guides involving two generations of Latin American lupus experts, both pediatricians and adults, and included experts from all PANLAR member countries. They were also reviewed by patient representatives.

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.

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.

Copyright of Revista de Medicina e Saúde de Brasília is the property of Revista de Medicina e Saude de Brasilia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The use of biological agents in chronic inflammatory diseases has increased, since its effectiveness has been demonstrated in several situations. Since these agents entered the market, tumor necrosis factor (TNF) antagonists are now widely prescribed for the treatment of several prevalent chronic inflammatory diseases. With the growth of immunobiological therapy, it was possible to notice an increase in the number of cases of Systemic Lupus Erythematosus induced by these drugs. Drug induced lupus erythematosus (DILE) is a syndrome of signs, symptoms and laboratory findings similar to idiopathic lupus erythematosus (SLE), triggered by the administration of certain drugs, disappearing after cessation of use. There is no completely clear mechanism in the pathogenesis of drug-induced lupus, and it is believed that different drugs can cause ILDD by different mechanisms. Thus, this study aimed to perform a literature review, with emphasis on TNF inhibitors inducing Systemic Lupus Erythematosus, and also the probable mechanisms involved in its pathophysiology. [ABSTRACT FROM AUTHOR]

Mixed connective tissue disease (MCTD) is a chronic autoimmune disorder consisting of a mixture of four diseases: systemic lupus erythematosus, systemic sclerosis, dermatomyositis/polymyositis, and rheumatoid arthritis. Because it is a combination of different autoimmune disorders its diagnosis is quite complex. Currently there are four combinations suggested by the following authors to establish an accurate diagnosis: Kasukawa, Alarcón-Segovia & Villareal, Kahn, and Appeboom & Sharp. It has been 46 years since Sharp reported the disease in 1972 and thus the purpose of this review was to investigate the evolution of the diagnosis of MCTD since then. PubMed and SciELO databases were used for this investigation. Because MCTD is an autoimmune disease that leads to the development of a chronic inflammatory condition, the STRING tool was used to allow the analysis of protein interaction. To date, there is no consensus as to what criterion should be used for a correct and efficient diagnosis of this disease. The low ratio of interactions observed from the STRING tool demonstrates that there is not yet enough data in the literature for establishing the binding between marker proteins and MCTD. [ABSTRACT FROM AUTHOR]

Objective: To present a case of nephrotic syndrome, secondary to syphilis, mimicking systemic lupus erythematosus. Case report: 26-year-old woman who developed painful oral ulcers, arthralgia and nephrotic syndrome, and the possibility of autoimmune disease was initially suggested. The results of the laboratory tests removed the possibility of systemic lupus erythematosus and confirmed the diagnosis of syphilis. Final considerations: The presence of nephrotic syndrome in a young female patient should trigger the investigation of previous sexual history, and syphilis is a mandatory possibility in this investigation, which can mimic SLE, delaying early diagnosis and effective treatment

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Lúpus eritematoso sistêmico é uma doença inflamatória crônica, que incide em maior frequência no sexo feminino, cujo diagnóstico acarreta várias alterações na vida da portadora. O objetivo deste estudo foi compreender o significado atribuído por mulheres ao fato de ter um diagnóstico de lúpus. Trata-se de uma pesquisa qualitativa fenomenológica, por considerar que ela permite a compreensão do fenômeno selecionado. Foram entrevistadas 10 mulheres portadoras de lúpus, residentes na cidade de Alfenas, no ano de 2010, com a seguinte questão norteadora: Como é para você, mulher, ter um diagnóstico de lúpus? Das falas, emergiram três categorias: autoimagem, relação familiar e restrições/incapacidades da doença. Foi possível perceber que, para a mulher ter um diagnóstico de lúpus, implica uma reorganização pessoal e familiar nos vários aspectos da vida: social, orgânico, psicológico, emocional e espiritual. ABSTRACT Systemic lupus erythematosus is a chronic inflammatory disease, which affects females more frequently than males. Diagnosis brings about a number of changes in the lives of women affected. This study aims at understanding women’s self-portrayal upon diagnosis of lupus. This is a phenomenological qualitative research, considering that it allows for the understanding of the phenomenon. Ten women with lupus, living in the city of Alfenas, Brazil, were interviewed in 2010, with the following question: How do you feel, as a woman, to have a diagnosis of lupus? Three categories emerged from their responses: self-image, family relation, and restrictions / disabilities generated by the disease. It was possible to realize that the diagnosis of lupus for women involves personal and family reorganization on a number of fronts, namely, social, organic, psychological, emotional, and spiritual. RESUMEN El lupus eritematoso sistémico es una enfermedad inflamatoria crónica, que afecta con mayor frecuencia las mujeres, cuyo diagnóstico trae varios cambios en la vida de ellas. El objetivo de este estudio fue comprender El significado atribuído al hecho de las mujeres tiener un diagnóstico de lupus. Esta es una investigac íon cualitativa fenomenológica, teniendo en cuenta que esto permite la comprensión de este fenómeno. Fueron entrevistados 10 mujeres con lupus, que viven en la ciudad de Alfenas-MG-Brasil en 2010, con la siguiente pregunta: ¿Cómo es para usted, tener un diagnóstico de lupus? A partir de los discursos, surgieron tres categorías: autoimagen, relación familiar y restricciones/ discapacidades de la enfermedad. Fue posible percibir que para la mujer tener un diagnóstico de lupus, implica una reorganización personal y familiar en los diversos aspectos de la vida social, orgánica, psicológica, emocional y espiritual.

Púrpura trombocitopênica trombótica (PTT) é uma alteração hematológica rara e com risco de morte, caracterizada por trombocitopenia, anemia hemolítica microangiopática e alterações neurológicas e/ou renais. A PTT foi descrita em raros pacientes com lúpus eritematoso sistêmico juvenil (LESJ) e, até onde se sabe, a prevalência dessa manifestação em uma população de lúpus pediátrico ainda não foi estudada. Assim, entre janeiro de 1983 e dezembro de 2010, revisamos os prontuários de 5.508 pacientes acompanhados na Unidade de Reumatologia Pediátrica do nosso hospital universitário. Foram identificados 279 (5,1%) casos de LESJ que preencheram os critérios de classificação do American College of Rheumatology. Dois destes (0,7%) apresentavam PTT, ambos no início do LESJ, e foram aqui descritos. Os dois pacientes tinham febre, anemia hemolítica microangiopática (com esquizócitos no sangue periférico) e trombocitopenia. O paciente do gênero masculino apresentava hemiparesia e proteinúria, e a paciente do gênero feminino tinha cefaleia persistente e hematúria. Ambos foram tratados com metilprednisolona endovenosa e plasmaferese quando do diagnóstico de PPT. Após tratamento, não houve recidiva da PTT, e hematócritos, contagens de plaquetas e níveis de desidrogenase lática permaneceram normais. Em conclusão, a PTT é uma rara e grave manifestação no início do LESJ. Os casos relatados reforçam a importância de um diagnóstico precoce e de uma terapia agressiva em pacientes com PTT, devido à sua alta morbidade.Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening hematological abnormality characterized by thrombocytopenia and microangiopathic hemolytic anemia, with neurological abnormalities and/or renal disease. TTP has been rarely reported in juvenile systemic lupus erythematosus (JSLE) patients and, to our knowledge, its prevalence in a paediatric lupus population has not been studied. Therefore, from January 1983 to December 2010, we reviewed the charts of 5,508 patients followed-up at the Paediatric Rheumatology Unit of our university hospital. We identified 279 (5.1%) JSLE cases that met the American College of Rheumatology classification criteria. Two (0.7%) of them had TTP, both at JSLE onset, and were described herein. Both patients had fever, microangiopathic hemolytic anemia (with schistocytes in blood smears), and thrombocytopenia. The male patient had hemiparesis and proteinuria and the female patient had persistent headache and hematuria. Both were treated with intravenous methylprednisolone and courses of plasma exchange therapy at TTP diagnosis. After treatment, TTP did not recur and their hematocrit, platelet count, and lactic dehydrogenase remained normal. In conclusion, TTP is a rare and severe manifestation at JSLE onset. The case reports reinforce the importance of early diagnosis and early aggressive treatment for patients with TTP, due to its high morbidity.

OBJETIVO: Examinar a associação entre a qualidade de vida e a distância percorrida no teste de caminhada de 6 minutos (6TC) em pacientes com lúpus eritematoso sistêmico (LES) na pré-menopausa, bem como comparar os resultados com controle saudáveis. MÉTODO: Foram pareadas por idade, características físicas e nível de atividade física (Questionário Internacional de Atividade Física: s-IPAQ) 25 pacientes com LES na pré-menopausa (18-45 anos) com baixa atividade da doença (SLEDAI médio: 1,52 ± 1,61) e 25 controles. Ambos os grupos não deviam estar envolvidos em atividade física regular por pelo menos 6 meses antes do estudo. Além da distância percorrida no 6TC (protocolo American Thoracic Society), foi avaliada a frequência cardíaca (FCpós) e a saturação de oxigênio (SpO2pós) pós-teste, e a percepção subjetiva de esforço de Borg (PSE/CR10). A qualidade de vida foi avaliada pelo Short Form Health Survey 36 (SF-36). RESULTADOS: Pacientes com LES apresentaram pior qualidade de vida, percorreram menor distância no 6TC (598 ± 45 m versus 642 ± 14 m; P < 0,001) e obtiveram maior PSE (6,28 ± 2 versus 5,12 ± 1,60; P < 0,05), FCpós (134 ± 15 bpm versus 123 ± 23 bpm; P < 0,05) quando comparadas aos controles. A qualidade de vida foi preditora significativa de 70% da distância percorrida no 6TC. CONCLUSÃO: Quando comparadas aos controles, as pacientes com LES percorrem menor distância no 6TC, o que foi associado a pior qualidade de vida.OBJECTIVE: To assess the association between quality of life and distance walked in the 6-minute walk test (6MWT) in Brazilian premenopausal patients with systemic lupus erythematosus (SLE) and compare their results with those of healthy controls. METHODS: Twenty-five premenopausal (18-45 years) patients diagnosed with low-activity SLE (mean SLEDAI: 1.52 ± 1.61) and 25 controls were matched for age, physical characteristics, and physical activity level (International Physical Activity Questionnaire/s-IPAQ). Both groups should not be involved in regular physical activity for at least six months before the study. The 6MWT distance (American Thoracic Society protocol), posttest heart rate (HRpost), posttest oxygen saturation (SpO2post) and the Borg scale of subjective perception of effort (SPE/CR10) were evaluated. The quality of life was assessed by use of the Short Form Health Survey 36 (SF-36). RESULTS: Patients with SLE had a significantly poorer quality of life, a shorter 6MWT distance (598 ± 45 m versus 642 ± 14 m, P < 0.001), and greater values of SPE/CR10 (6.28 ± 2.0 versus 5.12 ± 1.60, P< 0.05) and HRpost (134 ± 15 bpm versus 123 ± 23 bpm, P< 0.05) when compared with controls. The linear regression model suggested that quality of life was a significant predictor of 70% of the 6MWT distance. CONCLUSION: When compared with controls, patients with SLE walked a shorter distance in the 6MWT, which was associated with poorer quality of life.

A enteropatia perdedora de proteínas raramente pode ser observada em pacientes com lúpus eritematoso sistêmico. Essa situação clínica deve ser suspeitada quando houver hipoalbuminemia persistente, na presença de uma função hepática preservada, ingesta proteica adequada e ausência de proteinúria significativa. Descrevemos o caso de uma paciente de 48 anos com perda ponderal, derrames cavitários (ascite e derrame pleural) e edema de membros inferiores. O diagnóstico de lúpus foi firmado a partir da presença de linfopenia, proteinúria, FAN e autoanticorpos positivos (anti-Sm, anti-DNA e anti-Ro). Houve persistência de hipoalbuminemia mesmo com corticoterapia na dose de 1 mg/kg de peso, sendo diagnosticada enteropatia perdedora de proteínas por meio da cintilografia com albumina 99mTc. A melhora clínica e laboratorial da paciente veio após a associação da azatioprina com corticosteroide.Protein-losing enteropathy is rarely seen in patients with systemic lupus erythematosus. This clinical condition should be suspected in the presence of persistent hypoalbuminemia despite normal liver function, adequate protein intake, and no significant proteinuria. We report the case of a 48-year-old female with weight loss, cavity effusions (ascites and pleural effusion), and lower extremity edema. The diagnosis of lupus was established based on the presence of lymphopenia, proteinuria, ANA, and positive autoantibodies (anti-Sm, anti-DNA, and anti-Ro). Because hypoalbuminemia persisted even with corticosteroid therapy at the dose of 1 mg/kg, protein-losing enteropathy was diagnosed by use of Tc-99m albumin scintigraphy. After adding azathioprine to the treatment, the symptoms subsided and serum albumin levels improved.

A síndrome da encefalopatia posterior reversível (PRES) é uma entidade nova clinicamente caracterizada por cefaleia, alterações sensoriais, convulsões e perda visual. A patogenia da PRES ainda não foi esclarecida. A PRES pode estar associada a uma variedade de condições clínicas, principalmente hipertensão, insuficiência renal e terapia imunossupressora. Uma possível associação de doenças autoimunes com PRES foi recentemente sugerida. Aqui descrevemos dois casos de lúpus eritematoso sistêmico nos quais a PRES foi deflagrada por diferentes fatores.The posterior reversible encephalopathy syndrome (PRES) is a novel entity clinically manifested by headache, changes of sensorium, seizures, and visual loss. PRES pathogenesis has not been fully clarified. The entity can be associated to a variety of clinical conditions, mainly hypertension, renal insufficiency and immunosuppressive therapy. A possible link of autoimmune disorders with PRES has been recently hypothesized. We herein describe two cases of systemic lupus erythematosus whereby PRES was triggered by different factors.

OBJETIVOS: Determinar a frequência de anticorpos contra componentes da cromatina no lúpus eritematoso sistêmico juvenil (LESJ) e correlacionar a presença desses autoanticorpos com manifestações clínicas e atividade da doença. MÉTODOS: Os anticorpos anticromatina (anti-CHR), antinucleossomo (anti-NCS) e anti-dsDNA foram medidos em 175 indivíduos, incluindo 37 pacientes com LESJ ativo e 41 com doença inativa, 47 com doenças autoimunes não lúpicas, e 50 crianças saudáveis. Um teste ELISA in house foi desenvolvido com nucleossomos purificados a partir de timo de bezerro para determinar os anticorpos IgG e IgG3 anti-NCS. Anti-CHR e anti-dsDNA foram detectados por kits comerciais de ELISA (INOVA). RESULTADOS: Anticorpos anti-NCS e anti-CHR exibiram não só uma alta especificidade para LESJ, mas também uma frequência semelhante em LESJ ativo e inativo. Os níveis séricos de anti-CHR e IgG/IgG3 anti-NCS não diferiram entre LESJ ativo e inativo. Houve correlação entre o SLEDAI e os anticorpos anti-dsDNA, mas não com os anticorpos contra outros componentes da cromatina. Houve associação de anticorpos anti-dsDNA, anti-CHR e IgG/IgG3 anti-NCS com proteinúria e baixos níveis séricos de C4. Foram observados anticorpos anti-NCS em 14% dos pacientes com LESJ na ausência de anticorpos anti-dsDNA. CONCLUSÕES: Nossos dados indicam que os anticorpos anti-NCS e anti-CHR são marcadores diagnósticos relevantes para LESJ e parecem estar correlacionados com a atividade da nefrite lúpica no LESJ. O anticorpo IgG3 anti-NCS não parece ser mais relevante como marcador de atividade da doença ou nefrite ativa no LESJ em comparação ao anticorpo IgG anti-NCS.OBJECTIVES: To determine the frequency of antibodies to chromatin components in juvenile systemic lupus erythematosus (JSLE), and to correlate the presence of these autoantibodies with clinical manifestations and disease activity. METHODS: Anti-chromatin (anti-CHR), anti-nucleosome core particle (anti-NCS) and anti-dsDNA antibodies were measured in 175 individuals, including 37 patients with active JSLE and 41 with inactive disease, 47 non-lupus autoimmune disease patients (non-lupus AD), and 50 healthy children. An in-house ELISA was developed with purified nucleosome core particles from calf thymus to determine IgG and IgG3 anti-NCS antibodies. Anti-CHR and anti-dsDNA antibodies were detected by commercial ELISA kits (INOVA). RESULTS: Anti-NCS and anti-CHR antibodies exhibited high specificity for JSLE and similar frequency in active and inactive JSLE. Anti-CHR and IgG/IgG3 anti-NCS serum levels did not differ between active and inactive JSLE. SLEDAI correlated with anti-dsDNA antibodies but not with antibodies to other chromatin components. There was association of anti-dsDNA, anti-CHR and IgG/IgG3 anti-NCS antibodies with proteinuria and low C4 serum levels. Anti-NCS antibodies in the absence of anti-dsDNA were observed in 14% of the JSLE patients. CONCLUSIONS: Our data indicate that anti-NCS and anti-CHR antibodies are relevant diagnostic markers for JSLE and appear to be correlated with JSLE lupus nephritis activity. IgG3 isotype anti-NCS antibodies do not seem to be more relevant than IgG anti-NCS antibodies as markers of disease activity or active nephritis in JSLE.

Autoimmunity is characterized by tissue destruction that implicates functional damages caused by self-reactive cells that escape self-tolerance mechanisms. Autoimmune diseases can be initiated by viral infections and the study of the association between these viruses and autoimmunity has advanced the understanding of the molecular mechanisms involved in autoimmune diseases. The Human T-Cell Lymphotropic Virus Type I (HTLV-1) is a deltavirus that infects preferentially lymphocytes. Retrovirus particles like has been identified in patients with autoimmune diseases. Therefore this review had by objective approach the main aspects involving HTLV-1 with systemic lupus erythematosus and rheumatoid arthritis. Studies show that retroviruses can integrate their genetic material in host DNA, changing the expression gene profile related with apoptosis and immunologic system molecules. It’s known that HTLV-1 can cause different clinical manifestations in their careers and the mechanisms that triggers the HTLV-1 associated autoimmune diseases are not well known. Besides the perpetuation and marked production of pro-inflammatory cytokines, studies have demonstrated that both Th17 cells and T regulatory cells (Tregs) are involved in autoimmune diseases pathogenesis. Therefore the HTLV-1 viral particles recognized could be used as a risk marker in the development of autoimmune diseases.A autoimunidade é caracterizada pela destruição tecidual, que acarreta danos funcionais, causados por células autoreativas que escapam dos mecanismos de autotolerância. Doenças autoimunes podem ser iniciadas por infecções virais e o estudo da associação entre essas viroses e a autoimunidade tem possibilitado melhor conhecimento dos mecanismos moleculares envolvidos nas doenças autoimunes. O vírus linfotrópico de células T humano tipo 1 (HTLV-1) é um delta vírus que infecta preferencialmente linfócitos. Partículas semelhantes aos retrovírus foram identificadas em pacientes com doenças autoimunes. Portanto, esta revisão teve por objetivo abordar os principais aspectos envolvendo HTLV-1 com o lúpus eritematoso sistêmico e artrite reumatóide. Estudos demonstram que retrovírus podem integrar seu material genético no DNA do hospedeiro, alterando o perfil de expressão de genes relacionados a apoptose ou a moléculas do sistema imunológico. Sabe-se que o HTLV pode causar diferentes manifestações clínicas em seus portadores e os mecanismos pelos quais se desencadeiam as doenças autoimunes associadas ao HTLV-1 não estão bem esclarecidos. Além da perpetuação e produção acentuada de citocinas próinflamatórias, estudos têm demonstrado que tanto as células Th17 quanto as células T regulatórias (Tregs) estão envolvidas na patogênese de doenças autoimunes. Portanto o reconhecimento de partículas virais do HTLV-1 poderia ser utilizado como marcador de risco no desenvolvimento de doenças autoimunes.

Os autores revisaram a influência dos fatores nutricionais sobre o lúpus eritematoso sistêmico (LES) e abordaram uma alternativa complementar em seu tratamento. A autoimunidade e o processo inflamatório do LES estão relacionados à presença de dislipidemias, obesidade, hipertensão arterial sistêmica e síndrome metabólica, que devem ser adequadamente consideradas para diminuir o risco cardiovascular. Uma alimentação com moderado teor energético e proteico, mas rica em vitaminas, minerais (principalmente os antioxidantes) e ácidos graxos mono/poli-insaturados, pode promover ação benéfica protetora contra danos tissulares e supressão da atividade inflamatória, além de auxiliar no tratamento dessas comorbidades. A dietoterapia é uma abordagem promissora, e algumas recomendações podem oferecer melhor qualidade de vida aos pacientes com LES.The authors reviewed the influence of nutritional factors on systemic lupus erythematosus (SLE) and discussed an alternative treatment option. The autoimmunity and inflammatory process of SLE are related to the presence of dyslipidemia, obesity, systemic arterial hypertension, and metabolic syndrome, which should be properly considered to decrease cardiovascular risk. A diet with moderate protein and energy content, but rich in vitamins, minerals (especially antioxidants), and mono/polyunsaturated fatty acids can promote a beneficial protective effect against tissue damage and suppression of inflammatory activity, in addition to helping the treatment of those comorbidities. Diet therapy is a promising approach and some recommendations may offer a better quality of life to patients with SLE.

OBJETIVO: Avaliar a associação entre a presença de anticorpos antinucleossomo (anti-NCS) e a síndrome antifosfolipídica primária (SAFP) e o posterior desenvolvimento de lúpus eritematoso sistêmico (LES). MATERIAIS E MÉTODOS: Trinta e seis mulheres com o diagnóstico de SAFP foram avaliadas prospectivamente para manifestações de doenças reumáticas autoimunes e para a presença de anticorpos antifosfolípides, anticorpos antinucleares e anti-NCS/cromatina. RESULTADOS: Após um período médio de seguimento de 45,7 meses, anticorpos anti-NCS/cromatina foram detectados em apenas uma paciente (2,8%), que desenvolveu manifestações de LES tais como poliartrite, linfopenia, neurite óptica, lesões compatíveis com esclerose múltipla em substância branca cerebral e perfil de autoanticorpos altamente sugestivo de LES. CONCLUSÃO: A frequência de anticorpos anti-NCS/cromatina é baixa em pacientes com SAFP, e sua presença pode associar-se ao desenvolvimento de manifestações de LES.OBJECTIVE: To study the association of anti-nucleosome (anti-NCS) antibodies in primary antiphospholipid syndrome (APS) and the development of systemic lupus erythematosus (SLE) during follow-up. MATERIALS AND METHODS: Thirty-six women with primary APS were evaluated prospectively for clinical features of systemic autoimmune diseases and for the presence of antiphospholipid antibodies, antinuclear antibodies and anti-NCS/chromatin antibodies. RESULTS: After a mean follow-up period of 45.7 months, anti-NCS/chromatin antibodies were detected in only one patient (2.8%), who developed features of SLE including polyarthritis, lymphopenia, optic neuritis, multiple sclerosis-like lesions, and an autoantibody profile suggestive of SLE. CONCLUSION: The frequency of anti-NCS/chromatin antibodies in primary APS patients is very low, and they may be associated with the development of SLE manifestations.

INTRODUÇÃO: O presente estudo é uma análise da possível existência de distúrbios cognitivos associados a fibromialgia (FM), artrite reumatoide (AR) e lúpus eritematoso sistêmico (LES), e da influência das variáveis idade, escolaridade e sintomas psiquiátricos sobre esses distúrbios. MATERIAIS E MÉTODOS: Foram utilizados como amostra três grupos de pacientes, compostos por 13 indivíduos com FM, 13 com AR e 11 com LES, com idades entre 30 e 80 anos, encaminhados pelo Ambulatório de Reumatologia do Hospital Universitário de Brasília (HUB). O desempenho dos participantes da amostra nos testes neuropsicológicos de memória, linguagem, funções executivas e inventário neuropsiquiátrico foi analisado considerando-se os grupos conforme tipo de doença crônica, escolaridade e idade. Também foi realizada a comparação entre os pontos de corte de normalidade cognitiva em amostras populacionais com os desempenhos desses participantes. RESULTADOS: Os dados revelaram presença de distúrbios cognitivos associados às três patologias, porém com diferenças significativas entre os grupos. CONCLUSÃO: As variáveis estudadas (baixa escolaridade e idade elevada) revelaram-se associadas a diversos graus de declínio em diferentes funções cognitivas com os três grupos patológicos. Entretanto, os grupos FM e LES apresentaram médias de sintomas neuropsiquiátricos de ansiedade, irritabilidade e alucinações significativamente maiores que o grupo AR no inventário neuropsiquiátrico.INTRODUCTION: This study assesses the possible existence of cognitive disorder associated with chronic diseases [fibromyalgia (FM), rheumatoid arthritis (RA) and lupus (SLE)], and the influence of the variables age, educational level and psychiatric symptoms on those disorders. MATERIALS AND METHODS: The patients were referred by the Rheumatology Outpatient Clinic of the Hospital Universitário de Brasília (HUB), with ages ranging from 30 to 80 years, and were divided into the following three groups: FM, 13 patients; RA, 13 patients; and SLE, 11 patients. Their performance in the neuropsychological tests of memory, language, executive functions and neuropsychiatric inventory was assessed considering their type of chronic disease, educational level and age. In addition, the cutoff points of cognitive normality of population samples were compared with the patients' performances. RESULTS: The cognitive disorders were shown to be associated with the three diseases studied, but with significant differences between them. CONCLUSION: The variables studied (low educational level and advanced age) were associated with various degrees of impairment in the different cognitive functions in the three pathological groups. However, FM and SLE groups showed significantly higher means of the neuropsychiatric symptoms of anxiety, irritability and hallucinations than the RA group in the neuropsychiatric inventory.

Nos últimos anos têm sido descritos mediadores sintetizados no tecido adiposo, chamados adipocinas. Eles apresentam ação hormonal, regulando o apetite e o metabolismo da glicose, mas também atuam como citocinas, com efeitos sobre o sistema imune, incluindo efeitos na autoimunidade. As adipocinas mais importantes são: leptina, adiponectina, visfatina e resistina. Algumas delas vêm sendo estudadas nas doenças autoimunes reumáticas, particularmente no lúpus eritematoso sistêmico (LES) e na artrite reumatoide (AR). Estudos mostram níveis elevados de leptina e de adiponectina no LES, porém a correlação com a atividade da doença ainda é discutida. Na AR, os estudos também mostram aumento dos níveis de leptina e de adiponectina, observando-se correlação com a atividade da doença e a erosão articular; os resultados, porém, são conflitantes. Esta revisão descreve os papéis da leptina e da adiponectina no sistema imune e suas atuações no LES e na AR.In recent years, mediators synthesized in the adipose tissue, the so-called adipokines, have been described. They have a hormonal action, regulating appetite and glucose metabolism, but also act as cytokines with effects on the immune system, including effects on autoimmunity. The most important adipokines are leptin, adiponectin, resistin and visfatin, and some of them have been assessed in autoimmune rheumatic diseases, especially systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). Studies have shown high levels of leptin and adiponectin in SLE, but correlation with disease activity is questionable. In RA, studies have also reported increased levels of leptin and adiponectin, and correlation with disease activity and joint erosion, but the results are confl icting. This review describes the role of leptin and adiponectin on the immune system, as well as on SLE and RA.

O lúpus eritematoso sistêmico (LES) frequentemente cursa com serosites. Cerca de 16% dos pacientes lúpicos apresentam acometimento de pleura e pericárdio. O acometimento peritoneal, no entanto, é especialmente raro, ocorrendo clinicamente em pequeno grupo de pacientes. O presente estudo trata do caso de uma paciente de 47 anos com diagnóstico de lúpus discoide que evoluiu com manifestações sistêmicas da doença, caracterizadas por distensão e dor abdominal significativas, astenia, emagrecimento, sinais propedêuticos de ascite e diarreia aguda baixa e não invasiva. Foi realizada exaustiva investigação diagnóstica por meio de exames laboratoriais, de imagem, colonoscopia e análise do líquido ascítico. A investigação, além de descartar a possibilidade de etiologia infecciosa, neoplásica e hemodinâmica, também permitiu a confirmação de LES. Dessa forma, a hipótese de peritonite lúpica com ascite tornou-se viável. Foi introduzido tratamento com prednisona e cloroquina, com melhora substancial do quadroSerositis is commonly seen in systemic lupus erythematosus (SLE). Approximately 16% of patients with SLE have pleural or pericardial involvement. However, peritoneal involvement is extremely rare, and clinically seen in a small group of patients. This is the case report of a 47-year old female with discoid lupus who evolved with systemic manifestations of the disease, characterized by significant abdominal distension and pain, asthenia, weight loss, signs of ascites, and acute non-invasive diarrhea. Exhaustive diagnostic investigation was performed and included laboratory and imaging tests, colonoscopy, and analysis of the ascitic fluid. Besides ruling out the possibility of an infectious, neoplastic, and hemodynamic etiology, the investigation also allowed the confirmation of SLE. Thus, the hypothesis of lupus peritonitis with ascites became viable. The patient was treated with prednisone and chloroquine, with substantial improvement of her condition

INTRODUÇÃO: O papel imunorregulatório da vitamina D tem sido alvo de um crescente número de estudos em pacientes com lúpus eritematoso sistêmico (LES). Objetivos: Determinar os níveis séricos de 25-hidroxivitamina D3 [25(OH)D] em pacientes com LES e verificar a associação da insuficiência/deficiência de 25(OH)D com parâmetros clínicos e laboratoriais. MÉTODOS:Estudo de corte transversal, prospectivo, realizado no ambulatório de LES do Serviço de Reumatologia do Hospital das Clínicas da Universidade Federal de Pernambuco. Foram incluídos 78 pacientes portadores de LES e 64 voluntários (grupo de comparação) pareados por gênero e idade. RESULTADOS: Constatou-se insuficiência/deficiência de 25(OH)D em 45 (57,7%) pacientes com LES e em 25 (39%) indivíduos do grupo de comparação. Os níveis séricos médios de 25(OH)D foram 29,3 ng/mL (6,1-55,2 ng/mL) nos pacientes com LES e 33,12 ng/mL (15,9-63,8 ng/mL) no grupo de comparação; essa diferença é considerada estatisticamente significante (P = 0,041). Não houve diferença estatisticamente significante entre as médias de idade dos dois grupos. Não houve associação estatisticamente significante entre insuficiência/deficiência de 25(OH)D e tempo de diagnóstico, atividade de doença (SLEDAI > 6), fadiga, uso de corticosteroides e de antimaláricos e anti-DNA. CONCLUSÕES:Foi constatada alta prevalência de insuficiência/deficiência de 25(OH)D nos pacientes com LES (57,7%), com diferença estatisticamente significante em relação ao grupo de comparação. Não evidenciamos associação de insuficiência/deficiência de vitamina D com as variáveis clínicas e laboratoriais estudadas. Os autores enfatizam a importância da determinação dos níveis séricos de 25(OH)D em todos os pacientes com LES, independente de onde residam e do tempo de diagnóstico da doençaINTRODUCTION: The immunoregulatory role of vitamin D has been the object of a growing number of studies in patients with systemic lupus erythematosus (SLE). OBJECTIVES: To determine the serum levels of 25-hydroxyvitamin D3 [25(OH) D] in patients with SLE, and to assess the association of 25(OH)D insufficiency/deficiency with clinical parameters and laboratory tests. METHODS: Cross-sectional, prospective study performed at the SLE Clinic, Department of Rheumatology, Hospital das Clínicas, Universidade Federal de Pernambuco with convenience sampling, including 78 patients with SLE and 64 volunteers (comparison group), matched by gender and age. RESULTS: Insufficiency/deficiency of 25(OH)D was found in 45 (57.7%) patients with SLE and 25 (39%) individuals in the comparison group. The mean serum levels of 25(OH)D were 29.3 ng/mL (6.1-55.2 ng/mL) in patients with SLE and 33.12 ng/mL (15.9-63.8 ng/mL) in the comparison group, and this difference was statistically significant (P = 0.041). No statistically significant difference was observed between the mean ages of both groups. No statistically significant association was observed between 25(OH)D insufficiency/deficiency and the following: time to diagnosis; disease activity (SLEDAI > 6); fatigue; use of corticosteroids and antimalarials; and anti-DNA. CONCLUSIONS: High prevalence of 25(OH)D insufficiency/deficiency was found in patients with SLE (57.7%), with statistically significant difference as compared with the comparison group. No association of vitamin D insufficiency/deficiency was observed with the clinical variables and laboratory tests studied. The authors emphasize the importance of determining 25(OH)D serum levels in all patients with SLE, regardless of where they live and time to disease diagnosis

Estudos recentes sobre o torque teno vírus (TTV), gênero Anellovirus, permitiram construir a hipótese de que esse vírus pode ser um desencadeante ou tenha algum papel patogênico nas doenças reumáticas autoimunes. OBJETIVOS: Verificar a frequência da infecção pelo TTV em pacientes com lúpus eritematoso sistêmico (LES), e sua diversidade gênica, a existência de correlação entre a infecção pelo TTV e as manifestações clínicas do LES, sua evolução clínica e o perfil sorológico. PACIENTES E MÉTODOS: Foram obtidas 46 amostras de soro de pacientes com LES atendidos no Ambulatório de Reumatologia do Hospital Universitário de Campo Grande (NHU/FAMED/UFMS). Para os controles, utilizaram-se 46 amostras de soro de doadores de sangue. O DNA viral foi extraído das amostras utilizando o QIAamp DNA Blood Mini Kit (QIAGEN, Hilden, Alemanha), e amplificado utilizando a técnica de nested PCR. RESULTADOS: Foi encontrada positividade para o TTV em 17 (37%) dos pacientes lúpicos, e em apenas sete (15,2%) dos controles (teste z, P = 0,03). Não houve correlação entre a infecção pelo TTV, as manifestações clínicas, o perfil sorológico e a evolução clínica dos pacientes avaliados neste estudo. CONCLUSÃO: A presença do TTV nos pacientes com LES necessita ser mais bem compreendida a partir deste estudo inicialRecent studies on the torque teno virus (TTV), genus Anellovirus, have allowed formulating the hypothesis that TTV may trigger autoimmune rheumatic diseases or have some pathogenic role in them. OBJECTIVES: To determine the frequency of TTV infection in patients with systemic lupus erythematosus (SLE), the genetic diversity of TTV, the correlation between TTV infection and SLE clinical manifestations, and SLE clinical course and serological profile. PATIENTS AND METHODS:Serum samples were obtained from 46 SLE patients treated at the University-Affiliated Hospital of Campo Grande (NHU/FAMED/UFMS), Brazil. For controls, serum samples were obtained from 46 healthy volunteer blood donors. Viral DNA was extracted from samples using the QIAamp DNA Blood Mini Kit (QIAGEN, Hilden, Germany) and amplified using nested PCR. RESULTS: Positivity for TTV was found in 17 (37%) of SLE patients and in only seven (15.2%) of the controls (z test, P = 0.03). There was no correlation between TTV infection, SLE clinical manifestations, SLE clinical course, and the serological profile of the patients evaluated. CONCLUSION: Further studies on the presence of TTV in SLE patients are required

A síndrome do anticorpo antifosfolípide (SAF), mais comum em mulheres, manifesta-se clinicamente como trombose e/ou abortamentos de repetição. Anemia hemolítica autoimune e manifestações neurológicas, cardíacas e cutâneas são comuns. Relata-se o caso de um paciente do gênero masculino cuja manifestação inicial da doença foi gangrena em pavilhão auricular, e o diagnóstico de SAF se deu por meio de biópsia de pele do membro inferior, que mostrava vasculopatia trombótica, sem evidência de vasculite. Esse resultado é um dos dois critérios maiores que, associados a um critério menor, fecham o diagnóstico dessa doença. Discutem-se neste caso os possíveis diagnósticos diferenciais e como eles se diferenciam da doença em foco, além da importância que a biópsia teve no diagnóstico de SAF nesse indivíduo.Antiphospholipid syndrome (APS), more common in females, manifests clinically as thrombosis and/or recurrent fetal loss. Hemolytic autoimmune anemia and neurological, cardiac and cutaneous manifestations are common. This is the case report of a male patient whose first manifestation of the disease was gangrene of the auricle. The diagnosis of APS was established by biopsy of the lower limb skin, which showed thrombotic vasculopathy with no evidence of vasculitis. This is one of the two major criteria, which, along with a minor criterion, establishes the diagnosis of APS. Possible differential diagnoses are discussed. The importance of the biopsy in the APS diagnosis of this male patient is emphasized.

INTRODUÇÃO: O lúpus eritematoso sistêmico (LES) é uma doença autoimune com maior prevalência em mulheres. A maior incidência ocorre durante os anos reprodutivos, sugerindo que o estradiol tenha influência na apresentação clínica do LES. Anticorpos anticardiolipina (ac-ACL) estão relacionados com a síndrome do anticorpo antifosfolipídeo (SAF), mas podem estar presentes em pacientes com LES sem SAF, sendo relacionados com risco cardiovascular e nefrite. OBJETIVO: Determinar se a presença de ac-ACL está associada a alterações hormonais em uma amostra de mulheres com LES. MÉTODOS: Foram avaliadas 47 mulheres com LES de acordo com os critérios do American College of Rheumatology, com idade média de 30,8 ± 8,12 anos. Nenhuma fazia uso de anticoncepcional hormonal, e a atividade do LES foi estimada pelo índice de atividade da doença (SLEDAI). As pacientes foram estratificadas de acordo com a presença ou não de ac-ACL, e os níveis séricos de estradiol e prolactina foram determinados. RESULTADOS: Nove (19,1%) das 47 pacientes tiveram ac-ACL positivos. Idade, tempo de doença e o SLEDAI foram similares entre os grupos. No entanto, a mediana do estradiol foi menor no grupo com ac-ACL positivo [46,8 (21,0-72,1) pg/mL] com relação ao grupo com ac-ACL negativo [122,3 (64,8-172,7) pg/mL, P = 0,004]. CONCLUSÃO: Estes resultados sugerem, pela primeira vez, uma associação inversa entre ac-ACL e níveis de estradiol em pacientes pré-menopáusicas com LES. Considerando que tanto níveis reduzidos de estradiol endógeno quanto presença de ac-ACL estão associados a aterosclerose, este achado pode ser clinicamente relevante em predizer risco cardiovascular e/ou desenvolvimento de SAF no LES.INTRODUCTION: Systemic lupus erythematosus (SLE) is an autoimmune disease, with higher prevalence in women. An incidence peak occurs during the reproductive years, suggesting that estradiol may play a role in the clinical presentation of SLE. Anticardiolipin antibodies (ACA) are associated with antiphospholipid antibody syndrome (APLS), but can be found in patients with SLE without APLS, and relate to cardiovascular risk and nephrite. OBJECTIVE: This study aimed at assessing whether the presence of ACA is associated with hormonal changes in a sample of women with SLE. METHODS: Forty-seven women diagnosed with SLE according to the American College of Rheumatology criteria, aged 30.8 ± 8.12 years, were evaluated. None was on hormonal contraception, and their SLE activity was estimated using the SLE Disease Activity Index (SLEDAI). Patients were stratified, according to the presence or absence of ACA, and estradiol and prolactin levels were measured. RESULTS: Nine (19.1%) of 47 patients were positive for ACA. No differences were found between groups concerning age, duration of disease, and SLEDAI. In contrast, the median estradiol level was lower in the ACA-positive group [46.8 (21.0-72.1) pg/mL] than in the ACA-negative group [122.3 (64.8-172.7) pg/mL, P = 0.004]. CONCLUSION: These results suggest, for the first time, an inverse association between ACA and estradiol levels in premenopausal SLE patients. Considering that both lower endogenous estradiol levels and ACA positivity are related to atherosclerosis, our finding may be clinically relevant in predicting cardiovascular risk and/or APLS development in SLE.

INTRODUÇÃO: Estudos epidemiológicos brasileiros sobre o lúpus eritematoso sistêmico (LES) são bastante escassos e os dados existentes hoje são praticamente todos de literatura internacional. OBJETIVOS: Determinar a incidência e algumas características clínicas e laboratoriais de pacientes com LES em Cascavel, Paraná - Brasil. PACIENTES E MÉTODOS: Os dados foram coletados entre agosto de 2007 e julho de 2008 em todos os serviços de saúde do município que possuíam atendimentos na especialidade de Reumatologia: um hospital universitário, um ambulatório público e três clínicas privadas da cidade. RESULTADOS: Foram identificados 14 pacientes com diagnóstico de LES, resultando em uma incidência estimada de 4,8 casos/100.000 habitantes/ano. Todos os pacientes eram do sexo feminino, com média de idade de 41,5 anos. A faixa etária com maior incidência foi a de 30 - 39 anos e 92,8% apresentaram quatro ou mais dos 11 critérios do American College of Rheumatology (ACR) para o diagnóstico de LES. O tratamento farmacológico dos pacientes também foi avaliado e mostrou estar de acordo com o Consenso Brasileiro para o tratamento de LES. CONCLUSÃO: A incidência obtida em Cascavel/PR está próxima das incidências observadas em estudos internacionais.INTRODUCTION: Brazilian epidemiological studies on systemic lupus erythematosus (SLE) are scarce, and currently available data originate almost entirely from international literature. OBJECTIVES: To determine the incidence and some clinical and laboratory characteristics of patients with SLE in the municipality of Cascavel, state of Paraná, Brazil. PATIENTS AND METHODS: Data were collected from August 2007 to July 2008 in all health services of Cascavel providing health care in rheumatology: a university-affiliated hospital, a public outpatient clinic, and three private clinics. RESULTS: The study identified 14 patients diagnosed with SLE, which resulted in an estimated incidence of 4.8 cases/100,000 inhabitants/year. All patients were female, and the mean age was 41.5 years. The highest incidence of disease occurred between 30 and 39 years of age, and 92.8% of patients met at least four of the 11 American College of Rheumatology (ACR) criteria for diagnosis of SLE. The drug treatment of patients was also assessed and proved to be in accordance with the Brazilian Consensus for Treatment of SLE. CONCLUSION: The incidence obtained in the municipality of Cascavel is close to those reported in international studies.

A piomiosite tropical (PT) é uma doença infecciosa da musculatura esquelética, de etiologia bacteriana, incomum, e muitas vezes associada a situações de imunodeficiência. O envolvimento de musculatura pélvica profunda, como o músculo ilíaco, é uma condição ainda mais incomum. A associação de lúpus eritematoso sistêmico (LES) e PT é pouco relatada na literatura. Como o LES é uma situação de imunodepressão tanto pela doença em si como pelas medicações utilizadas no seu tratamento, esses pacientes tornam-se grupo de risco para o desenvolvimento de infecções, como PT. A infecção pelo HTLV está cada vez sendo mais identificada e associada a patologias nas quais autoimunidade está implicada, como é o caso do LES. Os autores descrevem um caso de PT de localização no músculo ilíaco em paciente portadora de LES, insuficiência renal crônica, em hemodiálise, além de portadora do vírus HTLV 1/2, internada no Hospital Heliópolis, em São Paulo, BrasilTropical pyomyositis (TP) is an unusual infectious disease of skeletal muscles, caused by bacteria, and often associated with immunodeficiency conditions. The involvement of deep pelvic muscles, such as the iliac muscle, is even rarer. The association of systemic lupus erythematosus (SLE) and PT is seldom reported in the literature. Because SLE involves a state of immunosuppression resulting from both the disease itself and its medicamentous treatment, SLE patients are at higher risk for developing infections, such as PT. Infection by HTLV 1/2 is increasingly identified and associated with autoimmune diseases, such as SLE. This is a case report of PT in the pelvic muscles of a female patient with SLE, chronic kidney failure, on hemodialysis, and HTLV1/2 infection, admitted to the Hospital Heliópolis, in the city of São Paulo, Brazil

O Lúpus Eritematoso Sistêmico (LES) é uma doença inflamatória crônica, multissistêmica, de natureza autoimune, de etiologia multifatorial, cujo diagnóstico baseia-se no quadro clínico somado a parâmetros laboratoriais. Este estudo teve como objetivo investigar medidas de avaliação em LES disponíveis na literatura, a partir do método da revisão integrativa de literatura. O levantamento bibliográfico foi realizado por meio de busca por artigos científicos indexados em bancos de dados de ciências da saúde em geral como Literatura Latino-Americana e do Caribe em Ciências da Saúde (LILACS), MEDLINE/Pubmed e Scientific Eletronic Library Online (SCIELO). Utilizaram-se os seguintes descritores: assessment and lupus, quality of life and lupus, damage and lupus, activity index and lupus e patient outcomes and lupus. As publicações selecionadas mostraram três domínios de avaliação da enfermidade: medidas de avaliação de atividade da doença, medidas de avaliação de danos causados pela doença e medidas de avaliação de qualidade de vida. O estudo mostrou que a percepção do estado de saúde e da qualidade de vida dos pacientes, bem como o impacto da doença e de seu tratamento, podem ser melhor avaliados com a utilização das medidas propostas pelos autoresSystemic lupus erythematosus (SLE) is a chronic inflammatory, multisystem, autoimmune and multifactorial disease, whose diagnosis is based on clinical and laboratorial parameters. This study aimed at investigating assessing measures in SLE available in the literature by using the method of integrative review of the literature. The bibliographic research was conducted through the search for scientific articles indexed in general health sciences databases, such as the Latin American and Caribbean Health Sciences (LILACS), MEDLINE/PubMed, and Scientific Electronic Library Online (SCIELO). The following descriptors were used: "assessment and lupus"; "quality of life and lupus"; "damage and lupus"; "activity index and lupus"; and "patient outcomes and lupus". The selected publications showed three domains of SLE assessment: assessing measures of disease activity; assessing measures of damage caused by the disease; and assessing measures of quality of life. The study showed that the patients' perception of health status and quality of life and the impact of the disease and its treatment can be better evaluated using the measures proposed by the authors

INTRODUÇÃO: Pacientes com lúpus eritematoso sistêmico (LES) podem apresentar alterações nutricionais desencadeadas pela doença ou pelo tratamento, e essas condições podem interferir no prognóstico. OBJETIVOS: Avaliar o estado nutricional, a atividade física e os aspectos associados em pacientes com LES. MÉTODOS: As características nutricionais, clínico-laboratoriais, sóciodemográficas e de tratamento de 170 mulheres com LES foram avaliadas, em estudo transversal. RESULTADOS: Pacientes com idade entre 18 e 60 anos foram incluídas, com média (DP) de idade e de duração da doença de 39,1 anos (10,0) e 9,9 anos (6,2), respectivamente. Duas (1,2%) pacientes foram classificadas como magreza grau I, 59 (34,7%) como eutróficas, 61 (35,9%) como sobrepeso, 37 (21,8%) como obesidade grau I, sete (4,1%) como obesidade grau II e quatro (2,4%) como obesidade grau III. Pacientes com sobrepeso e obesas apresentaram maior idade, menor escolaridade, maior índice de dano do LES, maior concentração sérica de complemento, maior frequência de hipertensão arterial e de diabetes mellitus, presença de insuficiência ovariana e menor frequência do uso de antimaláricos. Quanto à atividade física, 39 pacientes (22,9%) foram classificadas como inativas, 100 (58,8%) como insuficientemente ativas e 31 (18,2%) como ativas. Destas últimas, 13 (43,3%) se encontravam no grupo de eutróficos. CONCLUSÃO: A frequência de excesso de peso, nesta população, foi elevada e esteve associada a alguns fatores de risco tradicionais para doenças cardiovasculares e a alguns fatores de pior prognóstico do LES. Logo, incentivar o controle do peso deve fazer parte dos principais objetivos do tratamento de todo paciente com LESINTRODUCTION: Patients with systemic lupus erythematosus (SLE) may present nutritional changes triggered by disease or treatment, and these conditions may interfere with prognosis. OBJECTIVE: Assess the nutritional status, physical activity and associated factors in patients with SLE under treatment at the Service of Rheumatology of Hospital das Clínicas/Universidade Federal de Minas Gerais. METHODS: A cross-sectional study evaluating the nutritional status, clinical laboratory findings, sociodemographic, and treatment characteristics of 170 SLE female patients. RESULTS: Patients aged between 18 and 60 years were included. The mean (SD) age of patients and duration of SLE was 39.1 (10.0) and 9.9 (6.2) years, respectively. Two (1.2%) patients were classified as grade I underweight, 59 (34.7%) eutrophic, 61 (35.9%) as overweight, 37 (21.8%) as grade I obesity, seven (4,1%) as grade II obesity, and four (2,4%) as grade III obesity. Overweight and obesity were significantly associated with older age, lower education, higher SLE damage index, higher serum concentration of complement, higher incidence of hypertension and diabetes mellitus, presence of ovarian failure, and less frequent use of antimalarials Regarding physical activity, 39 patients (22.9%) were classified as inactive, 100 (58.8%) insufficiently active, and 31 (18.2%) active. Of the latter, 13 (43.3%) were in the eutrophic group. CONCLUSION: Excess weight was high in this population and associated with some traditional risk factors for cardiovascular disease and SLE poor prognosis. Therefore, encouraging weight control must be part of the main goals in treating SLE patients