Fascitis necrotizante asociada a esteatohepatitis en el curso del lupus eritematoso sistémico / Necrotizing Fasciitis Associated with Steatohepatitis in The Course of Systemic Lupus Erythematosus

Introducción: La fascitis necrotizante es un cuadro muy grave causado por una infección bacteriana de la piel y de tejidos blandos subcutáneos, cuya evolución es hacia la destrucción y necrosis de los tejidos en un corto espacio de tiempo; el lupus eritematoso sistémico es una enfermedad autoinmune de causa desconocida que quienes la padecen tienen una mayor probabilidad de contraer infecciones debido al mal funcionamiento del sistema inmunológico y/o los efectos secundarios causados por los medicamentos. Objetivo: Observar la importancia de un tratamiento rápido y eficaz de la fascitis necrotizante en un paciente con lupus eritematoso sistémico y esteatohepatitis no alcohólica. Presentación de caso: Se presentó el caso clínico de un paciente de 30 años con diagnóstico de lupus eritematoso sistémico que desarrolló de forma concomitante de fascitis necrotizante y esteatohepatitis no alcohólica. A pesar de un tratamiento adecuado, el paciente fue agresivo. Tuvo una estadía hospitalaria de 83 días, con una evolución desfavorable que conllevó a la muerte(AU)

Introduction: Necrotizing fasciitis is a very serious condition caused by a bacterial infection of the skin and subcutaneous soft tissues, whose evolution is towards the destruction and necrosis of the tissues in a short space of time; Systemic lupus erythematosus is an autoimmune disease of unknown cause that sufferers are more likely to contract infections due to poor immune system function and/or side effects caused by medications. Objective: To observe the importance of rapid and effective treatment of necrotizing fasciitis in a patient with systemic lupus erythematosus and non-alcoholic steatohepatitis. Case report: We report the clinical case of a 30-year-old patient diagnosed with systemic lupus erythematosus who concomitantly developed necrotizing fasciitis and nonalcoholic steatohepatitis. Despite adequate treatment, the patient was aggressive. The patient had a hospital stay of 83 days, with an unfavorable evolution that led to his death(AU)

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)

Repercusión del lupus eritematoso sistémico en la cavidad bucal / Influence of systemic erythematous lupus in the oral cavity

Introducción: El lupus eritematoso sistémico es una enfermedad autoinmune, inflamatoria y sistémica, de causa desconocida, que puede afectar al aparato estomatognático. Objetivo: Describir las manifestaciones bucomaxilofaciales en pacientes con lupus eritematoso sistémico. Métodos: Se realizó un estudio observacional, descriptivo, de serie de casos, de 25 pacientes con diagnóstico de la enfermedad, ingresados en el Servicio de Reumatología del Hospital Provincial Docente Clinicoquirúrgico Saturnino Lora Torres de Santiago de Cuba, durante el 2017. Resultados: Entre las lesiones intrabucales resultaron más frecuentes las periodontopatías, con 68,0 por ciento, y de las extrabucales, el eritema, con 40,0 por ciento. De igual forma, 10 pacientes presentaron alteración en la articulación temporomandibular, lo que representó 40,0 por ciento Conclusiones: Se pudo identificar una gran afectación en el complejo bucal a causa del lupus eritematoso sistémico. Al respecto, los estomatólogos desempeñan un importante papel en la detección de sus manifestaciones clínicas.

Introduction: The systemic erythematous lupus is an autoinmune, inflammatory and systemic disease, of unknown cause which can affect the stomatognatic aparatus. Objective: To describe the oral maxilofacial manifestations in patients with systemic erithematous lupus. Methods: An observational, series of cases descriptive study of 25 patients with diagnosis of the disease, admitted in the Rheumatology Service of Saturnino Lora Torres Teaching Provincial Clinical-Surgical Hospital in Santiago de Cuba was carried out during 2017. Results: Among the intraoral lesions there were the periodontopathies as the most frequent, with 68.0 percent, and of the extraoral lesions, the eritema, with 40.0 percent Likewise, 10 patients presented changes in the temporomandibular joint, what represented a 40.0 percent. Conclusions: A great damage could be identified in the oral complex because of the systemic erythematous lupus. In this respect, stomatologists plays an important role in the detection of its clinical manifestations.

Aumento de células T CD4+CD69+ e redução de células T reguladoras CD4+CD25+FoxP3+ em camundongos com Lúpus Eritematoso Sistêmico (LES) induzido por pristane / Increase of CD4+CD69+ T cells and reduction of CD4+CD25+FoxP3+ regulatory T cells in pristane-induced mice with systemic lupus erythematosus (SLE)

Introdução: O Lúpus Eritematoso Sistêmico (LES) é uma doença autoimune multissistêmica de etiologia complexa que envolve fatores ambientais, genéticos e hormonais. É caracterizada pela produção de autoanticorpos e mediadores inflamatórios, ativação e proliferação de células T autorreativas e perda da autotolerância imunológica. Em pacientes com LES, a expressão do receptor primário de ativação CD69 é aumentada e a de células T supressoras/reguladoras (Treg) CD4+CD25+FoxP3+ é reduzida. O CD69 é essencial para ativação de células T CD4 autorreativas enquanto que as células Treg são importantes na manutenção da autotolerância. Desta forma, células T tem um papel central na patogênese do LES, mas os mecanismos implicados na falência da autotolerância ainda não são elucidados, destacando a importância de estudos em modelos experimentais da doença, como o de LES-induzido por pristane. Objetivo: Quantificar células T CD4+CD69+ ativadas e Treg CD4+CD25+FoxP3+ no sangue, baço e LP de camundongos Balb/c LESinduzido por pristane no sentido de avaliar a falência de autotolerância neste modelo. Métodos: Analisamos 84 camundongos Balb/c fêmeas: 52 receberam por via intraperitoneal uma dose única de 0,5 ml de pristane e 32 a mesma dose de salina. Amostras de sangue, baço e LP dos camundongos eutanasiados foram coletadas 90, 120, 180 e 300 (T90, T120, T180 e T300) dias após a inoculação de pristane ou salina. Células mononucleares do sangue periférico (CMSP), do LP (CMLP) e esplenócitos foram obtidos por lise das hemácias seguida de lavagens com RPMI medium 1640 e centrifugação, e posteriormente criopreservadas até a avaliação por citometria de fluxo usando o aparelho Guava EasyCyteTM HT (Millipore). Para esta etapa, as células foram descongeladas, lavadas com RPMI medium 1640 e incubadas com anticorpos monoclonais dirigidos contra CD3, CD4, CD25, CD28, CD69, CTLA-4, FoxP3, CD14 e Ly6C (BD PharmingenTM). Os resultados foram expressos como média ± DP e teste de...

Introduction: Systemic Lupus Erythematosus (SLE) is multisystemic autoimmune disease with complex etiology that involves environmental, genetic and hormonal factors. Is characterized by auto-antibodies and inflammatory mediators production, autoreactive T cells activation and proliferation and loss of immunogenic autotolerance. In patients with SLE, expression of CD69 activation primary receptor is increased and the CD4+CD25+FoxP3+ suppressor/regulatory T cell (Treg) is reduced. CD69 is essential for activation of autoreactive CD4 T cells while Treg cells are important in autotolerance maintenance. In this way, T cells have a central role in the pathogenesis of SLE however, the mechanisms implied in the autotolerance failure are still not elucidated, highlighting the importance of studies in this disease's experimental models, such as pristane-induced SLE. Objective: Quantify activated CD4+CD69+ T cells and CD4+CD25+FoxP3+ Treg in blood, spleen and peritoneal lavage (PL) of Balb/c mice with pristane-induced SLE in order to evaluate autotolerance failure in this model. Methods: 84 female Balb/c mice were analyzed: 52 received a single intraperitoneal 0,5 ml dose of pristane and 32 the same dose of saline. Euthanized mice samples of blood, spleen and peritoneal lavage were collected 90, 120, 180 and 300 (T90, T120, T180 and T300) days after inoculation of pristane or saline. Mononuclear cells from peripheral blood (PBMC), PL (PLMC) and splenocytes were obtained by lysis of erythrocytes followed by washings with RPMI medium 1640 and centrifugation, subsequently criopreserved until evaluation by flow cytometry using the appliance GuavaEasyCyteTM HT (Millipore). For this step, cells were unfrozen, washed with RPMI medium 1640 and incubated with monoclonal antibodies against CD3, CD4, CD25, CD28, CD69, CTLA-4, FoxP3, CD14 and Ly6C (BD PharmingenTM). The results were expressed as mean ± SD and Mann-Whitney 11 test was used for statistical analysis, being considered...

Isquemia miocárdica extensa em mulher jovem com lúpus eritematoso sistêmico / Extensive myocardial ischemia among young woman with systemic lupus erythematosus

Paciente do sexo feminino, 24 anos, diagnosticada há seis anos com lúpus eritematoso sistêmico, apresentou dispneia aos médios esforços após quadro depré-eclampsia e edema agudo de pulmão. Foi realizada ecocardiografia que revelou trombo intracavitário no VEe discinesia, necessitando de internação para anticoagulação. A cintilografia tomográfica de perfusão miocárdica com 99mTc-sestamibi revelou defeitos de perfusão miocárdica compatíveis com área de isquemia estimada em 12 %. Discutem-se neste relato os aspectos clínicos das complicações cardiovasculares do lúpus eritematoso sistêmico.

Female patient, 24 years old, diagnosed six years ago with systemic lupus erythematosus, presented dyspnea on moderate exertion after preeclampsia and acute lung edema. Echocardiography disclosed an intracavitary thrombus in the LV with dyskinesia,requiring hospitalization for anticoagulation. Myocardial perfusion SPECT showed 99Tcm-sestamibi myocardial perfusion defects compatible with an area of ischemia estimated at 12%. This report discusses clinical aspects of cardiovascular complications resulting from systemic lúpus erythematous.

Neuropsychiatric lupus

Systemic lupus erythematosus [SLE] is a multisystem autoimmune disease. Involvement of the nervous system, either primary or secondary, is common in SLE, and 19 different neuropsychiatric clinical syndromes have been recognised in association with the disease. Several pathophysiological mechanisms have been implicated in the pathogenesis of neuropsychiatric SLE [NPSLE], including vasculopathy, autoantibodies, and cytokine-mediated tissue injury. Careful evaluation of the patient is required to rule out secondary causes before attributing the neurological symptoms to SLE. Treatment depends on the nature and severity of NPSLE syndrome

Insuficiência cardíaca secundária a lúpus eritematoso sistêmico / Heart failure secondary to systemic lupus erythematosus

O lúpus eritematoso sistêmico (LES) é uma doença sistêmica, de etiologia desconhecida e multifatorial. Está associado a diversas manifestações cardiovasculares como aterosclerose acelerada, pericardite, miocardite, valvulopatias, endocardite de Libman-Sacks, entre outras, contribuindo para a morbidade e mortalidade dos pacientes. Conclui-se, então, que tal colagenose deverá ser lembrada como causa de cardiomiopatia.

Systemic lupus erythematosus (SLE) is a multifactorial systemic disease of unknown etiology. It is associated with several cardiovascular manifestations, such as accelerated atherosclerosis, pericarditis, myocarditis, valvulopathies and Libman-Sacks endocarditis, amongothers, contributing to patient morbidity and mortality. It thus follows that this collagenosis should be recalled as a cause of cardiomyopathy.

Seasonal distribution of active systemic lupus erythematosus and its correlation with meteorological factors

OBJECTIVE: To explore the characteristics of seasonal distribution of active systemic lupus erythematosus (SLE) and the influences of meteorological factors including temperature and humidity on active systemic lupus erythematosus. METHODS: The characteristics of seasonal distribution of active SLE and its correlation with meteorological factors were retrospectively analyzed in 640 patients living in the city of Zhanjiang, China and had active SLE between January 1997 and December 2006. RESULTS: In winter, when there are weaker ultraviolet (UV) rays, the ratio of patients with active SLE to total inpatients was 3.89 percento, which is significantly higher than in other seasons with stronger UV rays, including 2.17 percento in spring, 1.87 0 in summer and 2.12 0 in autumn. The number of patients with active SLE had significant negative correlation with mean temperature and was not significantly related to mean humidity. CONCLUSION: Active SLE has the characteristics of seasonal distribution and is associated with temperature. The mechanism remains to be further studied.

Lúpus eritematoso sistêmico juvenil em adolescente com síndrome da imunodeficiência adquirida / Juvenile systemic lupus erythematosus in a adolescent with acquired immunodeficiency syndrome

O lúpus eritematoso sistêmico juvenil (LESJ) é uma doença inflamatória crônica, multissistêmica e autoimune. Algumas manifestações clínicas dessa condição são semelhantes às encontradas na síndrome da imunodeficiência adquirida (SIDA). A coexistência da SIDA com o LESJ é rara, especialmente na população pediátrica, sendo descritos na literatura pesquisada apenas cinco casos de pacientes com infecção congênita por HIV que desenvolveram essa enfermidade reumatológica, tendo como manifestação inicial a nefrite lúpica. Relata-se o caso de uma paciente de 14 anos, com diagnóstico de infecção pelo HIV aos 8 meses de idade, na qual se realizou diagnóstico de LESJ com base nos critérios diagnósticos do Colégio Americano de Reumatologia. Este relato tem a finalidade de descrever uma paciente com SIDA que, embora tenha desenvolvido LESJ em sua forma clássica e grave, evoluiu satisfatoriamente.

Systemic lupus erythematosus juvenile (SLEJ) is a multi-systemic, chronic inflammatory disease, and with autoimmune features. Some clinical manifestations of this disease are similar to those found inAcquired Immunodeficiency Syndrome (AIDS). Coexistence of AIDS with SLEJ is rare, especially in the pediatric population, being described in the literature just 5 patients with congenital HIV infection who developed this rheumatological condition, presenting lupus nephritis as the initial manifestation. We report the case of a 14 year old patient, diagnosed with HIV infection at 8 months of age, with signs and symptoms of SLEJ. This report aims to describe a female patient with AIDS who developed SLE in its classic and forms, but has evolved satisfactorily.

The role of apoptosis proteins and complement components in the etiopathogenesis of systemic lupus erythematosus

Systemic lupus erythematosus is a prototypical autoimmune disease characterized by the deregulation of T and B cells, tissue infiltration by mononuclear cells, tissue damage and the production of autoantibodies. There is a consensus that accelerated apoptosis of circulating lymphocytes and/or impaired clearance of apoptotic bodies may increase the amount of nuclear antigens presented to T lymphocytes. This process is accompanied by autoimmune responses that can lead to the development of lupus. The dysfunction of apoptosis may be a direct consequence of alterations in proteins/genes such as Fas, Bcl-2 and C1q. Increased expression of Fas antigen could intensify the exposure of hidden antigens. The overexpression of Bcl-2 protein might inhibit the removal of auto-reactive cells, and the lack of C1q could impair the clearance of self-antigens. The complete knowledge of the role of apoptosis components in the etiopathogenesis of lupus could lead to the development of new therapies targeting the apoptotic threshold, which could result in a more specific and effective disease response compared to global immunosuppression. This review summarizes the role of each component of the apoptotic process in the pathogenesis of lupus.

Lúpus eritematoso sistêmico / Systemic lupus erythematosus

O lúpus eritematoso sistêmico (LES) é uma doença inflamatória crônica, autoimune, que apresenta grande variação de etiopatogenia, manifestações clínico-laboratoriais e prognósticos. De modo geral, há predomínio de acometimento cutâneo e articular e alterações imunológicas com formação excessiva de múltiplos autoanticorpos.Seu diagnóstico se baseia em critérios clínicos e laboratoriais manifestados ao longo do tempo, com amplo diagnóstico diferencial, especialmente em fases iniciais. A apresentação clássica é de lesões cutâneas em mulheres jovens, no período fértil, mas há imensa variedade sindrômica. Sua etiologia ainda não está completamente elucidada, sendo provavelmente multifatorial e variável para diferentes indivíduos. Acredita-se que seja necessária a predisposição genética interagindo com fatores hormonais, ambientais e infecciosos, que levem à quebra da tolerância imunológica com formação de autoanticorpos contra-antígenos diversos, principalmente situados no núcleo celular. A evolução não é uniforme e são comuns os períodos de exacerbação, algumas vezes com reconhecidos desencadeantes - exposição solar, alterações hormonais, estresse físico e emocional. Pior prognóstico se associa ao acometimento renal, do sistema nervoso central, do cardiovascular e do hematopoiético, seja por evolução natural ou exigência de terapêutica mais agressiva. Os objetivos do tratamento são: restabelecimento da homeostase imunológica, controle de sintomas e preservação de órgãos evitando-se efeitos adversos da terapêutica medicamentosa prolongada. O vasto arsenal terapêutico inclui analgésicos, anti-inflamatórios não hormonais, corticosteroides, imunomoduladores e agentes biológicos. Com os progressos no tratamento tem ocorrido redução das lesões teciduais e aumento substancialmente da sobrevida, mas as complicações infecciosas ainda são preocupantes e, em pacientes com maior faixa etária, elevam-se as doenças cardiovasculares.

Periodontal disease and systemic lupus erythematosus activity

Because systemic lupus erythematosus (SLE) and periodontal disease share some pathogenetic similarities, a relationship between the two diseases might exist. This study aimed to assess SLE patients for the possible existence of a relationship between periodontal status and SLE disease activity. Fiveteen SLE patients had their disease activity index (SLEDAI), c-reative protein (CRP) serum levels and periodontal status determined. SLEDAI scores ranged from 0 to 11 and the CRP levels from 0.6 mg/l to 11.2 mg/l. The mean frequencies of periodontal sites with bleeding on probing and visible bacterial plaque were 8.79% (± 7.48) and 22.70% (± 26.32), respectively. The mean frequencies of sites with probing depths below 4 mm, between 4 and 6 mm and above 6 mm were 94.4% (± 10.87), 5.22% (± 10.07) and 0.38% (±0.92), respectively. Statistical analysis showed significant correlation between the frequency of sites with visible bacterial plaque and C3 serum levels. Although not statistically significant, correlations between the periodontal condition with SLEDAI scores and CRP serum levels were found. This study suggests that there is a relationship between periodontal status and SLE disease activity and between periodontal status and CRP serum levels.

Effects on prolactin secretion and binding to dopaminergic receptors in sleep-deprived lupus-prone mice

Sleep disturbances have far-reaching effects on the neuroendocrine and immune systems and may be linked to disease manifestation. Sleep deprivation can accelerate the onset of lupus in NZB/NZWF1 mice, an animal model of severe systemic lupus erythematosus. High prolactin (PRL) concentrations are involved in the pathogenesis of systemic lupus erythematosus in human beings, as well as in NZB/NZWF1 mice. We hypothesized that PRL could be involved in the earlier onset of the disease in sleep-deprived NZB/NZWF1 mice. We also investigated its binding to dopaminergic receptors, since PRL secretion is mainly controlled by dopamine. Female NZB/NZWF1 mice aged 9 weeks were deprived of sleep using the multiple platform method. Blood samples were taken for the determination of PRL concentrations and quantitative receptor autoradiography was used to map binding of the tritiated dopaminergic receptor ligands [³H]-SCH23390, [³H]-raclopride and [³H]-WIN35,428 to D1 and D2 dopaminergic receptors and dopamine transporter sites throughout the brain, respectively. Sleep deprivation induced a significant decrease in plasma PRL secretion (2.58 ± 0.95 ng/mL) compared with the control group (25.25 ± 9.18 ng/mL). The binding to D1 and D2 binding sites was not significantly affected by sleep deprivation; however, dopamine transporter binding was significantly increased in subdivisions of the caudate-putamen - posterior (16.52 ± 0.5 vs 14.44 ± 0.6), dorsolateral (18.84 ± 0.7 vs 15.97 ± 0.7) and ventrolateral (24.99 ± 0.5 vs 22.54 ± 0.7 µCi/g), in the sleep-deprived mice when compared to the control group. These results suggest that PRL is not the main mechanism involved in the earlier onset of the disease observed in sleep-deprived NZB/NZWF1 mice and the reduction of PRL concentrations after sleep deprivation may be mediated by modifications in the dopamine transporter sites of the caudate-putamen.

A New Onset of Systemic Lupus Erythematosus Developed After Bee Venom Therapy

Lupus is a systemic autoimmune disease of an unknown origin, and systemic lupus erythematosus (SLE) can be triggered by numerous stimuli. Bee venom therapy is an alternative therapy that is believed to be effective for various kinds of arthritis. We present here a case of a 49-year-old female who experienced a new onset lupus after undergoing bee venom therapy, and this looked like a case of angioedema. The patient was successfully treated with high dose steroids and antimalarial drugs. We discuss the possibility of bee venom contributing to the development of SLE, and we suggest that such treatment should be avoided in patients with lupus.

Inmunopatogenia del lupus eritematoso sistémico, Parte I: factores predisponentes y eventos iniciales / Pathogenesis of systemic lupus erythematosus, Part I: initial predisposing factors and events

El Lupus Eritematoso Sistémico (LES) es una enfermedad autoimmune sistémica caracterizada por la producción de autoanticuerpos y una diversidad de manifestaciones clínicas. A pesar de que su etiología es desconocida, existen susceptibilidades en los genes relacionados con la presentación antigénica, diferenciación y sobrevida de linfocitos B, activación linfocitaria, proliferación y apoptosis, producción de citoquinas y barrido de restos apoptóticos, que asociados a factores ambientales y hormonales determinan que la enfermedad se manifieste. En los eventos iniciales de su inmunopatogenia destaca el rol de una apoptosis defectuosa que determina, por una parte, la liberación de autoantígenos modificados hacia la circulación y, por otra, la sobrevida de linfocitos T y linfocitos B autorreactivos, con la generación de anticuerpos predominantemente contra antígenos nucleares, aunque los autoanticuerpos pueden estar dirigidos contra cualquier otro antígeno. La multiplicidad de manifestaciones clínicas se debe a la ubicuidad de estos antígenos.

Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disease that is characterized by the production of autoantibodies and a great diversity of clinical manifestations. Although its etiology is unknown, there is susceptibility to genes associated to the antigenic presentation, differentiation and survival of B lymphocytes, lymphocyte activation, proliferation, apoptosis, cytokines production and clearance of apoptotic bodies, which in association with environmental and hormonal factors determines the manifestation of the disease. The initial events in the pathogenesis of SLE include defects in apoptosis that produce, on one hand, the liberation of modified autoantigens into circulation and, on the other hand, the survival of T and autoreactive B lymphocytes with the generation of antibodies, predominantly against nuclear antigens, though the autoantibodies may be directed against any other antigen. The multiplicity of clinical manifestations is due to the ubiquity of these antigens.

Curso de Reumatologia: aula 4: manifestações gastrointestinais nas colagenoses / Course of Rheumatology: class 4: gastrointestinal manifestations in collagenosis

O termo colagenose, surgido em 1942, é hoje substituído por doenças difusas do tecido conjuntivo, que se constituem em um grupo de moléstias que afetam o tecido conjuntivo, rico em colágeno. São enfermidades que podem comprometer vários sistemas e múltiplos órgãos, sendo, portanto, de caráter sistêmico. O objetivo desta aula é discutir as manifestações gastrointestinais e sua abordagem terapêutica. Serão focadas algumas doenças difusas do tecido conjuntivo, a saber: esclerose sistêmica, lúpis eritematoso sistêmico, dermatopolimiosite e Sjõgren.

The term "collagen diseases" was first introduced by Klemperer in 1942, and is currently been called connective tissue diseases. All the organ and systems are both affected by them, so that's why it can be called systemic diseases. The aim of this lesson is to discuss gastrointestinal manifestations and its therapeutic approach, while it will be detailed the connective tissue diseases like systemic sclerosis, systemic lupus erythematosus, polymyositis/dermatomyositis and Sjõgren's syndrome.

Doença de Kikuchi-Fujimoto / Kikuchi-Fujimoto disease

A doença de Kikuchi-Fujimoto é caracterizada por febre e linfadenopatia, geralmente cervical. Esta doença acomete principalmente mulheres jovens. Pode ser confundida com linfoma, metástase de adenocarcinoma e tuberculose. Relatamos dois casos da doença de Kikuchi-Fujimoto. No primeiro caso, uma paciente de 28 anos havia tratado tuberculose há um ano e apresentava quadro clínico e histológico compatível com a doença de Kikuchi-Fujimoto. A segunda paciente, de 58 anos, recebeu tratamento inicialmente para granulomatose de Wegener e, posteriormente, para tuberculose. O exame histopatológico com estudo imunohistoquímico permitiu estabelecer o diagnóstico da doença de Kikuchi-Fujimoto nos dois casos. Após o diagnóstico definitivo, ambas foram tratadas sintomaticamente e melhoraram clinicamente dentro de um mês. Posteriormente, a segunda paciente desenvolveu lúpus eritematoso sistêmico.

Kikuchi-Fujimoto disease is characterized by fever and lymphadenopathy, usually localized in the cervical region. This disease principally affects young females. It can be confused with lymphoma, adenocarcinoma metastasis and tuberculosis. We report two cases of Kikuchi-Fujimoto disease. In the first case, a 28-year-old female had been treated for tuberculosis one year prior and presented with a clinical and histological profile compatible with Kikuchi-Fujimoto disease. The second patient, a 58-year-old female, initially received treatment for Wegener's granulomatosis and, subsequently, for tuberculosis. Histopathological examination followed by immunohistochemical analysis confirmed the diagnosis of Kikuchi-Fujimoto disease in both cases. After the definitive diagnosis had been made, both patients were treated symptomatically, and both presented clinical improvement within one month. Subsequently, the latter patient developed systemic lupus erythematosus.

Supervivencia en pacientes cubanos con lupus eritematoso sistémico, influencia de las características iniciales de la enfermedad / Survival of Cuban patients with systemic erythematous lupus, influence of the initial characteristics of the disease

Introducción: El Lupus eritematoso sistémico (LES) es una enfermedad autoimmune con un riesgo elevado de desarrollar complicaciones. Objetivo: Conocer las tasas de supervivencia, factores pronósticos según características presentes al inicio de la enfermedad y causas de muerte de los pacientes con LES. Métodos: Se estudió una cohorte de 244 pacientes con diagnóstico de LES, atendidos en el Servicio de Reumatología del Hospital Hermanos Ameijeiras entre septiembre de 1991 y agosto del 2006. Se recogieron características sociodemográficas, clínicas, de laboratorio e histológicas presentes al inicio de la enfermedad. Se realizó un análisis estadístico para relacionarlas con la supervivencia. Se establecieron las causas de muerte, según los datos clínicos y al examen post morten, se estimó la supervivencia según el tiempo de diagnóstico y la fecha de última noticia, mediante el método de Kaplan - Meier. Resultados: Fallecieron 34 de los 244 pacientes, las causas más frecuentes de muerte fueron las infecciones, en el 32,3 por ciento de los casos .Las tasas de supervivencia a los 5, 10 y 15 años fueron de 93, 82,5 y 70 por ciento, respectivamente. Las variables asociadas a menor supervivencia fueron la trombocitopenia (p=0,005), antiDNA elevado (p=0,037), fracción C3 baja (p=0,000), fracción C4 baja (p=0,000), Addis de 2 h alterado (p=0,004), proteinuria de mas de 0,5 g (p=0,024), creatinina elevada (p=0,000) y filtrado glomerular disminuido (p=0,000). Fueron factores independientes asociados a baja supervivencia la trombocitopenia, la fracción C3 del complemento baja y creatinina sérica elevada. Conclusiones: La supervivencia de pacientes cubanos con LES es a los 5 años similar a la de varios estudios de países desarrollados, variables asociadas a baja supervivencia dependen de características propias de la enfermedad.

Introduction: The systemic erythematous lupus (SEL) is an autoimmune disase with an elevated risk for developing complications. Objective: To know the survival rates, prognostic factors according to the characteristics present at the onset of the disease and causes of death of the patients with SEL. Methods: A cohort of 244 patients with diagnosis of SEL that received attention at the Rheumatology Service of Hermanos Ameijeiras Hospital from September 1991 to August 2006 were studied. Sociodemographic, clinical, lab and histological characteristics observed at the onset of the disease were collected. A statistical analysis was made to relate them to survival. The causes of death according to clinical data and to the postmortem examination were determined, and survival was estimated in accordance with the diagnosis and the last notice date by Kaplan-Meier's method. Results: 34 of the 244 patients died. The most common cause of death was infection in 32.3 percent of the cases. The survival rates at 5, 10 and 15 years were 93, 82.5 and 70 percent, respectively. The variables associated with a lower survival were thrombocytopenia (p=0.005), elevated antiDNA (p=0.037), low C3 fraction (p=0.000), low C4 fraction (p=0.000), Addis at 2 h altered (p=0.004), proteinuria over 0.5 g (p=0.024), elevated creatinin (p=0.000) and reduced glomerular filtrate (p=0.000). The independent factors associated with low survival were thrombocytopenia, low complement C3 fraction and elevated serum creatinin. Conclusions: the survival of Cuban patients with SEL at 5 years is similar to the one reported by diverse studies conducted in developed countries. The variables associated with low survival depend on the own characteristics of the disease.

Estudo da densidade mineral óssea em pacientes com lúpus eritematoso sistêmico antes e após uso de glicocorticóides / Study of the bone mineral density in patients with systemic lupus erythematosus before and after glucocorticoid treatment

OBJETIVO: Avaliar a variabilidade da densidade mineral óssea em pacientes com lúpus eritematoso sistêmico, antes do início e após 30 e 60 dias do início de glicocorticoterapia. MATERIAIS E MÉTODOS: Estudo efetuado em 15 mulheres em pré-menopausa com lúpus eritematoso sistêmico encaminhadas para realização de densitometria óssea e que fariam uso de corticosteróides logo após o exame. RESULTADOS: Demonstrou-se que a densidade mineral óssea, em g/cm², na coluna lombar reduziu-se significativamente entre a análise prévia ao uso e a análise em 60 dias de uso da medicação, mas no colo femoral não houve diferença significativa. CONCLUSÃO: Concluiu-se que o uso de corticoterapia em pacientes com lúpus eritematoso sistêmico, em curto prazo, reduz significativamente a densidade mineral óssea na coluna lombar, não somente em longo prazo como descrito anteriormente por outros autores.

OBJECTIVE: To evaluate the bone mineral density variability in patients with systemic lupus erythematosus, before the beginning and at 30 and 60 days from the beginning of glucocorticoid therapy. MATERIALS AND METHODS: Fifteen premenopausal women with systemic lupus erythematosus were referred for bone densitometry before being submitted to glucocorticoid therapy. RESULTS: It was demonstrated that the bone mineral density in g/cm² in the lumbar spine presented a significant decrease in the span between the first analysis previously to the beginning of the glucocorticoid therapy and the third analysis after 60 days under therapy. On the other hand no significant difference was found in the femoral neck. CONCLUSION: The authors conclude that, at short term, rather than only at long term as previously reported by other authors, corticotherapy in patients with systemic lupus erythematosus results in a decrease in the bone mineral density in the lumbar spine.