Mortality from systemic erythematosus lupus in Brazil: evaluation of causes according to the government health database / Mortalidade por lúpus eritematoso sistêmico no Brasil: avaliação das causas de acordo com o banco de dados de saúde do governo

Abstract Objective: To characterize the causes of mortality in patients with systemic lupus erythematosus (SLE) in Brazil between 2002 and 2011. Methods: An exploratory ecological study of a time series using data from the Mortality Information System of DATASUS, the Department of the Unified Health System (Brazil's National Health System). Results: Brazil's SLE mortality rate was 4.76 deaths/105 inhabitants. The mortality rate was higher in the Midwest, North and Southeast regions than in the country as a whole. There were 6.3% fewer and 4.2% more deaths than expected in the Northeast and Southeast regions, respectively. The mean age at death was 40.7 ± 18 years, and 45.61% of deaths occurred between the ages of 20 and 39. Incidence was highest in women (90.7%) and whites (49.2%). Disorders of the musculoskeletal system and connective tissue were mentioned as an underlying cause of death in 77.5% of cases, and diseases of the circulatory system and infectious and parasitic diseases were also noted in fewer cases. SLE was mentioned as an underlying cause of death in 77% of cases, with no difference between the Brazilian regions (p = 0.2058). The main SLE-related causes of death were, sequentially, diseases of the respiratory and circulatory systems and infectious and parasitic diseases. Conclusions: This study identified a need for greater control of risk factors for cardiovascular diseases and a better understanding of the pathogenesis of atherosclerosis in SLE. Infectious causes are still frequent, and management should be improved, especially in the early stages of the disease.

Resumo Objetivo: Caracterizar as causas de mortalidade em pacientes com lúpus eritematoso sistêmico (LES) no Brasil entre 2002 e 2011. Métodos: Estudo ecológico exploratório de uma série cronológica com dados do Sistema de Informações sobre Mortalidade do Departamento de Informática do Sistema Único de Saúde (Datasus). Resultados: A taxa de mortalidade por LES no Brasil foi de 4,76 mortes/105 habitantes. A taxa de mortalidade foi maior nas regiões Centro-Oeste, Norte e Sudeste do que no país como um todo. Houve 6,3% menos e 4,2% mais mortes do que o esperado nas regiões Nordeste e Sudeste, respectivamente. A média de idade ao óbito foi de 40,7 ± 18 anos e 45,61% dos óbitos ocorreram entre 20 e 39 anos. A incidência foi maior nas mulheres (90,7%) e nos brancos (49,2%). Os distúrbios do sistema musculoesquelético e do tecido conjuntivo foram mencionados como a causa subjacente de morte em 77,5% dos casos; também foram observadas doenças do sistema circulatório e infecciosas e parasitárias, embora em menor frequência. O LES foi mencionado como a causa subjacente de óbito em 77% dos casos, sem diferença entre as regiões brasileiras (p = 0,2058). As principais causas de morte associadas ao LES foram, em ordem, doenças dos sistemas respiratório e circulatório e doenças infecciosas e parasitárias. Conclusões: Este estudo identificou a necessidade de maior controle dos fatores de risco para doenças cardiovasculares e uma melhor compreensão da patogênese da aterosclerose no LES. As causas infecciosas ainda são frequentes e o manejo deve ser melhorado, especialmente nos estágios iniciais da doença.

Morbimortalidad cardiopulmonar en pacientes con lupus eritematoso sistémico / Cardiopulmonary morbidity and mortality in patientswith systemic lupus erythematosus

Los pacientes con diagnóstico de lupus eritematoso sistémico (LES) presentan unaelevada morbi-mortalidad asociada a las manifestaciones cardiopulmonares que sedesarrollan durante la evolución de la enfermedad. En este sentido, se han descrito dospicos de incidencia de mortalidad de esta enfermedad inmunológica. El primer pico estárelacionado a la actividad de la enfermedad y se observa durante los tres primeros añosposteriores al diagnóstico. El segundo pico está relacionado principalmente a lascomplicaciones cardiopulmonares y se presenta entre los 4 y 20 años posteriores aldiagnóstico. Si bien la mortalidad temprana ha disminuido gracias a un mayorconocimiento de la fisiopatología de la enfermedad y al uso de terapiasinmunosupresoras, la mortalidad tardía presenta un aumento progresivo de sufrecuencia a pesar del avance en el manejo terapéutico. El estudio de estascomplicaciones se ha convertido en un tema de gran interés debido a su efecto negativoen el pronóstico de los pacientes que las presentan. Algunos estudios epidemiológicossugieren que los pacientes con LES tienen un mayor riesgo cardiovascular al sercomparados con la población sana. En este sentido, se ha descrito que estos presentanun mayor riesgo de desarrollar eventos cardiovasculares (infarto agudo del miocardio,accidente cerebro vascular y enfermedad arterial periférica) en comparación con lapoblación general. De forma asociada, se ha objetivado que la presencia demanifestaciones pleuro-pulmonares (pleuritis lúpica, neumonitis, hemorragia pulmonar,embolismo pulmonar e hipertensión pulmonar) aumenta la morbimortalidad de lospacientes con LES. El conocimiento adecuado de las complicaciones cardiopulmonaresen los pacientes con diagnóstico de lupus eritematoso sistémico permitirá realizar unmanejo individualizado y por lo tanto más eficaz, permitiendo disminuir la morbilidadasociada a las citadas complicaciones...

Patients with the diagnosis of systemic lupus erythematosus (SLE) have an elevatedmorbidity and mortality from cardiopulmonary complications that develop during theevolution of the disease. Considering the incidence of the mortality in this immunologic disease, two peaks have been described. The first one is related to the immune activityitself and it is observed in the first three years the diagnosis. The second peak is relatedto the cardiopulmonary complications and it is observed after 4 to 20 years afterdiagnosis. Although, early mortality has diminished due to better knowledge of thephysiopathology of the disease and to the use of immunosuppressive drugs, latemortality has a progressive increment despite advances in the therapeutic management.There is a great interest in the study of these complications due to the negative impacton the prognosis of the SLE patients. Some epidemiologic studies suggest that patientswith SLE have a higher cardiovascular risk than the healthy population. Indeed, theyhave greater risk of developing cardiovascular events (acute myocardial infarction,cerebrovascular accidents, and peripheral artery disease) in comparison to the generalpopulation. The presence of pulmonary complications (pleuritic effusion, pneumonitis,pulmonary hemorrhage, pulmonary embolism, pulmonary hypertension) increases themortality in SLE patients. The proper knowledge of the cardiopulmonary complicationsin SLE patients will provide an individual and more effective management allowing adecrease in morbidity and mortality...

Descripción de las características epidemiológicas, estancia hospitalaria, frecuencia de mortalidad y causas de hospitalización en pacientes con lupus eritmatoso sistémico internados en un hospital nacional / Description of the epidemiological features, hospital stay frequency of mortality and causes of hospitalization in patients with systemic erythematosus lupus in a national hospital

OBJETIVO. Describir las características epidemiológicas, el tiempo de estancia hospitalaria, la frecuencia de mortalidad y las principales causas de hospitalización de Los pacientes con lupus eritematoso sistémico (LES) en Los servicios de internamiento del Hospital Nacional Cayetano Heredia (HNCH), durante el periodo (2002-2012). MATERIAL Y METODOS. Se realice un estudio primario, descriptivo-retrospectivo, que analizo la población de pacientes de 14 o más años con LES en los servicios de hospitalización de Medicina Interna del HNCH. La información se obtuvo del departamento de Estadística. RESULTADOS. Se evaluó 582 registros de hospitalizaciones, con respecto a 364 registros de pacientes, con un total de 9463 días-hospital, una relación mujer y hombre de 7,9/1, la edad del total de registros presenta una mediana de 30 años (IQT: 23-43). La estancia hospitalaria tuvo una mediana de 12 días (IQT: 5-22). Del total de registro- paciente, 155 (42,6 %) correspondían a causas infecciosas y 209 (57,4 %) correspondían a causas no infecciosas como causa de hospitalización. La frecuencia de mortalidad fue de 8,5 % con respecto al total de registro-paciente. Las etiologías más frecuentes que registraron condición de egreso fallecido fueron choque séptico pulmonar (1 J) Y accidente cerebrovascular (5). La condición de sepsis tuvo relación con mortalidad (OR = 2,77y P = 0,007). CONCLUSION. Él LES en Los servicios de hospitalización del HNCH tiene un comportamiento similar a 10 descrito en la literatura con respecto a estancia hospitalaria, mortalidad y causas de hospitalización; la condición de sepsis y el compromiso de los sistemas cardiaco, pulmonar y neurológico estuvieron relacionados a mortalidad.

OBJECTIVES. To describe the epidemiological characteristics, length of hospital stay, mortality rate and main causes of hospitalization of patients with systemic lupus erythematosus (SLE) in the inpatient services of Cayetano Heredia National Hospital (HNCH) during the period from 2002 to 2012. MATERIAL AND METHODS. A retrospective, descriptive study in which primary patient population of 14 or more years was analyzed SLE inpatient HNCH Internal Medicine was conducted. The information was obtained from the Department of Statistics. RESULTS. It was evaluated /582 records of hospitalizations with respect to 364 patient records, finding a total of 9 463 days-hospital, a female/male ratio of 7,9/1 was found, the age of the total records presented a median of 30 years (IQT: 23-43).The hospital stay was a median of 12 days (IQT: 5-22). Of the total log-patient, 155 (42,6%) related to infectious causes and 209 (57,4 %) were non-infectious causes as cause of hospitalization. The mortality rate was 8,5 % relative to the total log-patient. The most common etiologies of deceased recorded discharge condition, septic shock werelung (11), stroke (5), alveolar hemorrhage (4). The condition sepsis mortality was related to mortality found an OR 2,77 and P 0,007. CONCLUSIONS. The LES in the inpatient services HNCH has described a similar behavior ill 'the literature regarding hospital stay, mortality and causes of hospitalization; the condition of sepsis and the commitment of the cardiac, pulmonary, and neurological systems were related to mortality.

Systemic Lupus Erythematosus in Childhood.

Systemic lupus erythematosus (SLE) is a multisystem, autoimmune disorder characterised by the formation of antibodies to cellular components. In spite of high morbidity and mortality of childhood SLE our patient responded well to the therapy.

Outcomes and organ dysfunctions of critically ill patients with systemic lupus erythematosus and other systemic rheumatic diseases

Our objective was to compare the pattern of organ dysfunctions and outcomes of critically ill patients with systemic lupus erythematosus (SLE) with patients with other systemic rheumatic diseases (SRD). We studied 116 critically ill SRD patients, 59 SLE and 57 other-SRD patients. The SLE group was younger and included more women. Respiratory failure (61 percent) and shock (39 percent) were the most common causes of ICU admission for other-SRD and SLE groups, respectively. ICU length-of-stay was similar for the two groups. The 60-day survival adjusted for the groups’ baseline imbalances was not different (P = 0.792). Total SOFA scores were equal for the two groups at admission and during ICU stay, although respiratory function was worse in the other-SRD group at admission and renal and hematological functions were worse in the SLE group at admission. The incidence of severe respiratory dysfunction (respiratory SOFA >2) at admission was higher in the other-SRD group, whereas severe hematological dysfunction (hematological SOFA >2) during ICU stay was higher in the SLE group. SLE patients were younger and displayed a decreased incidence of respiratory failure compared to patients with other-SRDs. However, the incidences of renal and hematological failure and the presence of shock at admission were higher in the SLE group. The 60-day survival rates were similar.

Systemic lupus erythematosus among Jordanians: a single rheumatology unit experience

To study the characteristics of Systemic Lupus Erythematosus in Jordan and compare results with other Arab countries in the region. This descriptive study was conducted on 50 Jordanian patients with Systemic Lupus Lrythematosus attending the rheumatology clinic at King Hussein Medical Center between 2000 and 2007. King Hussein Medical Centre is one of the main referral hospitals in Jordan. Data was collected from patients, relating to the various disease manifestations and mode of presentation, age at disease onset, disease duration, and family history of Systemic Lupus Erythematosus. Statistical analysis was performed using simple descriptive and bivariate statistics, such as mean, median, range and Chi square. Forty-four females and six male patients were included. Female to male ratio was 7.3:1 with median age of 22 years [range 14-48]. Mean disease duration was 43 months [range 12-90 months]. Clinical manifestations of Systemic Lupus Erythematosus in descending order were, musculoskeletal [90%], cutaneous and oral ulcers [85%], renal complications [50%], neuropsychiatric [45%], haematological [40%], pulmonary complications [30%], and cardiac complications in 15% of cases. Secondary anti-phospholipid antibody syndrome [APS] occurred in 30% of cases. Three patients died from severe chest infection. Another patient died from massive sagittal sinus thrombosis and severe hydrocephalus. A comparison between our findings and published Systemic Lupus Erythematosus studies from four Arab countries revealed no significant clinical differences. Systemic Lupus Erythematosus is an important rheumatological disease with serious consequences including morbidity and mortality. It behaves in a similar way to Systemic Lupus Erythematosus in other Arab countries. Renal, pulmonary and neurological complications are the main cause of morbidity. Chest infection is a major cause of mortality

Mortalidad en los pacientes con lupus eritematoso sistémico que ingresaron a una unidad de cuidados intensivos / Mortality in the patients with systemic lupus erythematosus that entered to an intensive care unit

Objetivo. Determinar la mortalidad de los pacientes con lupus eritematoso sistémico (LES) que ingresaron a una unidad de cuidados intensivos (UCI). Material y Métodos. Se realizó un estudio de serie de casos, retrospectivo, en el que se evaluó todos los primeros ingresos de los pacientes con LES a la UCI del Hospital Nacional Arzobispo Loayza de Lima, durante el periodo enero 2003-diciembre 2007. En cada caso, se obtuvo los puntajes SLEDAI, APACHE II y SOFA de ingreso, para luego determinar su poder pronóstico de mortalidad de los pacientes. Resultados. De un total de 32 casos, 28 (92,9 por ciento) fueron mujeres y 4 varones (7,1 por ciento), con edad promedio de 71,63 mas o menos 12,40 años. La mortalidad de los pacientes fue de 46,4 por ciento. Los puntajes fueron: SLEDAI,14,7 mas o menos 5,2 (el 96,4 por ciento dentro del rango de actividad severa); APACHE II, 17,3 mas o menos 6,4; y, SOFA, 7,5 mas o menos 3,3. Sobre la base de una mediana de 7 mas o menos 8,5 de días UCI de seguimiento, el promedio de sobrevida fue en general a los 7 y 14 del 69,3 por ciento y 48,8 por ciento, respectivamente. Solo el puntaje SOFA fue significativamente asociado al evento muerte (6,3 mas o menos 3,8 puntos vs. 8,8 mas o menos 3,5 puntos, p = 0,0424); dicionalmente, luego del análisis multivariado, SOFA fue el único puntaje que fue significativamente asociado a la sobrevida de los pacientes (HR =1,27; IC95 por ciento: 1,03-1,56, p = 0,0267). Conclusiones. El puntaje SOFA fue mejor que los puntajes APACHE II y SLEDAI para predecir la mortalidad de los pacientes con LES que ingresaron a la UCI.

Objective. To determine the mortality rate in patients with systemic lupus erythematosus who entered to the Intensive Care Unit. Material and Methods. A retrospective study serialcase study was done in SLE patients who were admitted to the ICU in the Hospital Arzobispo Loayza, Lima, during January 2003-December 2007. In each case, it was collected SLEDAI, APACHE II and SOFA scores at ICU admission. Results. From a total of 32 cases, 28 (92,9 per cent 9 women and 4 (7,1 per cent) male, and an average age 71,63 more or less 12,40 year-old. The mean scores SLEDAI 14,7 more or less 5,2 (96,4per cent within the range of severeactivity; APACHE II 17.3 more or less 6,4; and, SOFA 7,5 more or less 3,3 (media more or less SD). Based on a mean 7 more or less 8,5 ICU-days monitoring, the mortality rate was 69,3 per cent and 48,8 per cent at 7 and 14 ICU-days, respectively. Only score SOFA predicted mortality better (6,3 mor or less3,8 vs. 8,8 per cent 3,5, p = 0,0424). Conclusions. The mortality rate of SLE patients admitted to the ICU could be predicted better bythe SOFA score than the APACHE II and SLEDAI-2K scores.

Perfil dos pacientes com lúpus eritematoso sistêmico, internados na unidade de terapia intensiva de um hospital universitário de Fortaleza / Characteristics of patients with systemic lupus erythematosus admitted to the intensive care unit in a brazilian teaching hospital

OBJETIVOS: Face à demanda de nosso serviço, buscamos descrever as características e a evolução dos pacientes com lúpus eritematoso sistêmico (LES) internados na unidade de terapia intensiva do Hospital Universitário Walter Cantídio. MÉTODOS: Os pacientes foram, retrospectivamente, caracterizados quanto aos dados demográficos, tempo de diagnóstico da doença, disfunções orgânicas e exames laboratoriais à admissão, suportes terapêuticos usados durante a internação, tempo de internação hospitalar prévio à admissão, tempo de permanência na unidade, reinternações e desfecho evolutivo. Foram avaliados os escores Systemic Lupus Erythematosus Disease Activity (SLEDAI) e Acute Physiological and Chronic Health Evaluation II (APACHE II) à admissão, a mortalidade prevista e a razão de mortalidade padronizada. RESULTADOS: No período de novembro de 2003 a outubro de 2006, 1.052 pacientes foram admitidos à UTI, 50 (4,75 por cento) dos quais com LES. Houve predomínio do sexo feminino (88,2 por cento), com média de idade de 30,3 ± 12,8 anos. A mediana do tempo de diagnóstico da doença foi de 67 meses. As disfunções mais prevalentes à admissão foram: renal (70,6 por cento), cardiovascular (61,8 por cento), respiratória (55,9 por cento) e neurológica (55,9 por cento). As principais disfunções motivadoras da admissão na unidade de terapia intensiva foram: respiratória (38,2 por cento), cardiovascular (29,4 por cento) e neurológica (29,4 por cento). Os tratamentos mais utilizados foram: hemocomponentes (44,1 por cento), fármacos vasopressores (41,2 por cento), ventilação mecânica (35,3 por cento) e diálise (23,5 por cento). A média do SLEDAI foi 15 ± 12,2 pontos e a do APACHE II foi 19,3 ± 6,8 pontos, com mortalidade prevista de 37,6 por cento. Registrou-se óbito de 20,6 por cento após 48 horas na unidade de terapia intensiva e 8,8 por cento, com menos de 48h. A razão de mortalidade padronizada foi 0,78. Os pacientes com APACHE II maior que 18 pontos, com ...

OBJECTIVES: Due to the high incidence in our service, we did object on this study describe the features and outcome of patients with systemic lupus erythematosus (SLE) admitted to the intensive care unit of Walter Cantídio University Hospital METHODS: Patients were restrospectively characterized according to demography parameters, time of diagnosis of SLE, organ dysfunction and laboratorial parameters at admission, supportive therapies during their stay, length of stay in the hospital before admission, length of stay in the unit, readmission to the unit and outcome. We also evaluated Systemic Lupus Erythematosus Disease Activity (SLEDAI) score, Acute Physiology and Chronic Health Evaluation II (APACHE II) score, expected mortality and standardized mortality ratio. RESULTS: From November 2003 to October 2006, 1,052 patients were admitted to the intensive care unit. Fifty patients had SLE and were included in this retrospective study. Of the 50 patients with SLE admitted to the ICU, 88.2 percent were female. The mean age was 30.3 ± 12.8 years. The median time of diagnosis of SLE was 67 months. The most common organ dysfunctions were renal (70.6 percent), cardiovascular (61.8 percent), respiratory (55.9 percent) and neurological (55.9 percent). The main reasons for admission to the ICU were respiratory (38.2 percent), cardiologic (29.4 percent) and neurological (29.4 percent) dysfunctions. Among the intensive care therapies, 44.1 percent of the patients needed blood products, 41.2 percent vasopressor agents and 35.3 percent mechanical ventilation, 23.5 percent dialysis. The mean SLEDAI score was 15.0 ± 12.2. The mean APACHE II score was 19.3 ± 6.8, with a predicted mortality rate of 37.6 percent. The actual mortality rate in ICU was 29.4 percent, with 8.8 percent before 48 hours. The standardized mortality ratio was 0.78. Patients with APACHE II > 18, with more than 3 acute organ involvements, leukopenia (< 4000 cells/mm3) and gastrointestinal ...

Lupus eritematoso sistémico en la unidad de cuidados intensivos de medicina del Hospital Nacional Cayetano Heredia / Systemic lupus erythematosus in the medical intensive care unit (ICU) at Hospital Nacional Cayetano Heredia

Objetivos: Describir las características clínicas y factores pronósticos del Lupus Eritematoso Sistémico (LES) en la Unidad de Cuidados Intensivos (UCI)de Medicina del Hospital Nacional Cayetano Heredia (HNCH). Material y métodos: Colección retrospectiva de hallazgos clínicos y de laboratorio de historias clínicas de pacientes con LES en la UCI del HNCH en el período de 1999 - 2004. Se hizo estudio estadístico descriptivo y análisis univariado para variables continuas y discretas. Se calculó el score APACHE II y se evaluó su significado pronóstico. Resultados: Hubo 26 pacientes con LES de los cuales se encontraron historias clínicas de 21 de ellos. La frecuencia de LES en la UCI del HNCH es de 1.49 por ciento, es la patología reumática más frecuente en UCI. La mortalidad fue de 57.69 por ciento. El motivo de admisión más frecuente fue insuficiencia respiratoria (52.4 por ciento), mayormente debido a causa infecciosa. Las variables que se asociaron significativamnete a mortalidad fueron: velosidad de sedimentación globular (VSG) alta, anemia severa, hipoalbuminemia severa, la relación presión arterial de oxígeno entre la fracción inspirda de oxígeno (PaFi)<200 al ingreso. El score APACHE II en el grupo de fallecidos fue significativamnete más alto (19.9) (p<0.01). La insuficiencia renal aguda requirió hemodiálisis y la necesidad de ventilación mecánica se asociaron significativamente a mayor mortalidad. Conclusiones: El LES en la UCI del HNCH tiene una alta mortalidad mayor que la reportada en estudios previos. Los factores de mal pronóstico fueron VSG alta, anemia severa, hipoalbuminemia severa, PaFi<200, score APACHE II alto,, necesidad de hemodiálisis y de ventilación mecánica.

Risk factors associated with the death of patients hospitalized for juvenile systemic lupus erythematosus

We assessed the risk factors associated with death in patients hospitalized for juvenile systemic lupus erythematosus (JSLE) and evaluated the autopsy reports. A total of 57,159 hospitalizations occurred in our institution from 1994 to 2003, 169 of them involving 71 patients with JSLE. The most recent hospitalization of these patients was evaluated. Patients were divided into two groups based on mortality during hospitalization: those who survived (N = 53) and those who died (N = 18). The main causes of hospitalization were JSLE activity associated with infection in 52 percent and isolated JSLE activity in 44 percent. Univariate analysis showed that a greater risk of death was due to severe sepsis (OR = 17.8, CI = 4.5-70.9), systemic lupus erythematosus disease activity index (SLEDAI) ³8 (OR = 7.6, CI = 1.1-53.8), general infections (OR = 6.1, CI = 1.5-25), fungal infections (OR = 5.4, CI = 3.2-9), acute renal failure (OR = 5.1, CI = 2.5-10.4), acute thrombocytopenia (OR = 3.9, CI = 1.9-8.4), and bacterial infections (OR = 2.3, CI = 1.2-7.5). Stratified analysis showed that severe sepsis and SLEDAI ³8 were not confounder variables. In the multivariate analysis, logistic regression showed that the only independent variable in death prediction was severe sepsis (OR = 98, CI = 16.3-586.2). Discordance between clinical diagnosis and autopsy was observed in 6/10 cases. Mortality of hospitalized JSLE patients was associated with severe sepsis. Autopsy was important to determine events not detected or doubtful in dead patients and should always be requested.

Cardiovascular involvement in systemic lupus erythematosus: an autopsy study of 27 patients in India.

BACKGROUND: Although cardiovascular disease (CVD) is recognized as a leading cause of death in patients with systemic lupus erythematosus (SLE) in western countries, there is hardly any data regarding Indian subjects with SLE. AIMS: To determine the incidence of cardiac abnormalities and vascular lesions at autopsy and to assess their contribution to the mortality in patients with SLE. SETTINGS AND DESIGN: Retrospective retrieval of reports of autopsies performed on 35 patients with SLE over a 11 year period and analysis of 27 cases with cardiac and/or vascular lesions. MATERIALS AND METHODS: Gross and microscopic features in 27 autopsies were analyzed with special attention to the heart and the vasculature of all organs. Findings were correlated with clinical features and ante-mortem investigations. Their contribution towards mortality was assessed. RESULTS: Valvar lesions were the commonest cardiac lesions noted with non-bacterial thrombotic endocarditis in nine (33.33%), valvar thickening in two (7.41%), Libman-Sacks endocarditis and infective endocarditis in one (3.70%) each. Myocarditis and myocardial scarring were seen in 10 (37.03%) and seven (25.92%) cases, respectively. Fibrinous pericarditis was noted in seven (25.92%). Thromboses/embolism, vasculitis and severe coronary atherosclerosis were seen in nine (33.33%), five (18.52%) and one (3.70%) subjects, respectively. Renal disease [13, 48.14%] and cardiovascular manifestations [8, 29.62%] were the leading causes of death in our patient population. CONCLUSION: CVD contributes significantly to the mortality in patients with SLE in India. It is second only to renal disease in this regard.

Mortality study in Puerto Ricans with systemic lupus erythematosus

OBJECTIVE: To determine the most common causes of death among Puerto Ricans with systemic lupus erythematosus. METHODS: Chart and record review of all deaths related to SLE complications from 1960 to 1994 at the University of Puerto Rico Hospital. RESULTS: Out of 662 patients diagnosed with SLE 161 (24) died. There were 151 (94) females and 10 (6) males. Mean duration of disease was 11.5 years. Mean age at death was 37 years. The primary causes of death were infection in 44 (27), uremia in 42 (26), cardiovascular complications in 33 (20), central nervous system complications in 18 (11), and pulmonary complications (other than infectious pneumonia) in 12 (7). CONCLUSIONS: The most common causes of death in SLE were infections and renal disease.

Mortalidad en pacientes con Lupus Eritematoso Sistémico e infecciones. Asociación con neutropenia y fiebre / Mortality in patients whith systemic lupus erythematosus and infections. Associated with neutropenic and fever

Con el objetivo de analizar la mortalidad y sus principales causas se evaluó a los pacientes con Lupus Eritematoso Sistémico (ACR82) que presenta-ron infecciones durante un período de estudio de 12 años. Se evaluaron en particular aquellos con neutropenia y fiebre. Ocurrieron 442 episodios de infecciones en 216 pacientes. 21 pacientes (10 por ciento) presentaron N y F en 28 episodios. La edad promedio fue de 35.6ñ9.6, el tiempo medio de evolución del LES fue de 5.8+- 2 (2 m.-25 a.). El lupus estuvo activo en el 93 por ciento) de los pacientes. El grado de actividad fue de 10.5ñ5.9 en neutropenicos febriles (N y F) vs 5.06.t4.7 en no-neutropenicos febriles (no-N y F) (p-< 0.0001). El tratamiento con ciclofósfamida i.v. se asoció con el desarrollo de neu-tropenia y fiebre (p<- 0.0002). El promedio de neutrofilos fue de 408/mm ñ185.6 (200-806). La duración media de la neutropenia fue de 10.9 días (245 días). Durante el episodio infeccioso fallecieron 38 pacientes (17.6 por ciento), 12/21 con N y F: (57 por ciento) y 26/195 (13 por ciento) no -N y F (p-< 0.00001). Las causas mas frecuentes de muerte fueron: infecciones en 21/38 casos (55.26 por ciento) , el compro-miso multiorgánico del lupus en 6 (16 por ciento), tromboembolismo pulmonar en 5 pasos (13 por ciento) . Predominó la mortalidad por infecciones en los pacientes con N y F (66.6 por ciento) vs. los no-N y F (50 por ciento) pero sin diferencia estadísticamente significativa.

Lúpus eritematoso sistêmico juvenil: manifestações clínicas, laboratoriais e evolutivas em 59 pacientes / Juvenile systemic lupus erythematosus: clinical manifestations, laboratory data, and evolution in 59 patients

O intuito deste trabalho foi avaliar as manifestações clínicas, dados laboratoriais e as causas de óbito de 59 pacientes (48F/11M) com lúpus eritematoso sistêmico (LES), cujos sintomas se iniciaram até os 16 anos de idade. Para tal realizaou-se um estudo restrospectivo com revisão dos prontuários de pacientes matriculados nos ambulatórios de Pediatria e Reumatologia do Hospital das Clínicas da Unicamp durante o período de 1979 a 1995. Os pacientes com lúpus eritematoso discóide (LED), induzido por drogas e neonatal não foram estudados. Analisaram-se os pacientes individualmente e também quando classificados em três grupos, conforme a idade de início das manifestações. Todos os pacientes preenchiam pelo menos quatro dos critóerios classificatórios do American College of Rheumatology (ACR) para LES. Os exames subsidiários foram realizados pelos laboratórios de Patologia Clínica (LPC) e de Imunologia Clínica, Alergia e Reumatologia (Licar) do HC-Unicamp. Os acometimentos clínicos mais frequentes foram: articular (91,5 por cento), renal (71,1 por cento), rash malar (61 por cento), alopecia (61 por cento), febre (61 por cento) e fotossensibilidade (52,5 por cento). As alterações laboratoriais mais detectadas foram: fator antinúcleo (FAN) (94,9 por cento), célula LE (71,1 por cento), consumo de complemento (65,3 por cento), anti-DNA (63,4 por cento), hematúria (62,7 por cento e proteinúria (61 por cento). A mortalidade foi de 23,7 por cento. As causas dos 14 óbitos (9F/5M) foram: processo infeccioso em oito (57,1 por cento), acidente vascular cerebral (AVC) em cinco (35,7 por cento) e insuficiência renal em um (7,2 por cento). O início das manifestações até os 14 anos e o acometimento renal foram indicadores de pior prognóstico

Lupus eritomatoso sistémico: manifestaciones neuropsiquiátricas / Manifestation neuropschyatric: erithematosus systemic lupus

Las manifestaciones neuropsiquiátricas se consideran una manifestación común del Lupos Eritematoso Sistémico (LES). La psicosis y las convulciones son criterios utilizados, por la Asociación Americana de Reumatismo, para definir la enfermedad. Sin embargo la sintomatología no psicótica es frecuentemente atribuida a la enfermedad. En nuestro estudio prospectivo, de 86 pacientes que ingresaron a los servicíos de Medicina 1 y psquiatría del Hospital Vargas de Caracas, realizamos un seguimiento por catorce años, con el objetivo de encontrar los trastornos neuropsiquiátricos presentes y si este podía ser aplicado por la actividad multisistématica del LES o por factores no orgánicos. Treinta (30) pacientes presentaron manifestaciones neuropsiquiátricas (34,8 por ciento) veintitrés (23) pacientes evidenciaron alteraciones psiquiátricas (76,6 por ciento) y ocho (8) mostraron hallazgos de disfunción neurológica (23,4 por ciento). Clínicamente se observó: 15 casos de delirio (34,9 por ciento) 6 casos con disfunciones mayores (14 por ciento), con disfunciones menores (51,1 por ciento); las manifestaciones neurológicas fueron: 4 pacientes con convulciones (50 por ciento), 2 con alteración del movimiento (25 por ciento), 1 con hemiparesia, (12,5 por ciento) y un caso con parálisis del séptimo nervio craneal (12,5 por ciento). El (86,7 por ciento) de los pacientes mostraron estas manifestaciones durante los primeros tres años de diagnóstico del LES. Los antecedentes personales fueron positivos en 15 pacientes (65,2 por ciento) y negativos en 8 (34,7 por ciento); X2= 3,73, p>0,05. RR=1,64. Los antecedentes familiares fueron positivos en 12 pacientes (52,2 por ciento) y negativos en 11 (47,8 por ciento); X2=4,10, p<0,05,RR=1,64. La evolución clínica fue favorable en 20 pacientes (66,6 por ciento). Diez pacientes fallecieron (33,4 por ciento) por insuficiencia renal o sepsis

Causa de muerte en lupus eritomatoso sistémico: estudio de necropsias en 27 pacientes del Hospital San Juan de Dios

Basados en el análisis de los registros de necropsias de pacientes que fallecieron con Lupus eritomatoso sistémico en el Hospital San Juan de Dios de Santafé de Bogotá en los últimos 16 años, se pretende conocer las causas de mortalidad de esta entidad entre nosotros. La primera de estas causas fue relacionada con el compromiso directo de la enfermedad, siendo la nefropatía la asociación más frecuente. Le sigue en frecuencia las infecciones, notándose como lo indican otras series, la presencia de gérmenes oportunistas

Systemic lupus erythematosus in Indian patients: prognosis, survival and life expectancy.

BACKGROUND: There have been several studies on survival patterns in Indian patients with systemic lupus erythematosus but it is still not clear as to which factors at diagnosis predict survival outcome. The impact of specific organ involvement, and of disease activity itself, needs to be studied further. METHODS: We conducted a non-concurrent prospective study of 98 lupus patients between 1981 and 1993. The clinical symptoms, signs and investigation results at onset, and at subsequent visits, were abstracted from the case notes. A systemic lupus erythematosus disease activity index (SLEDAI) was constructed at the initial presentation and for each subsequent visit. Patients not attending for at least 6 months were traced by post. The quantitative data from the SLEDAI was used to construct a Markov chain mathematical expression designed to predict life expectancy. RESULTS: The cumulative percentage survival at 1, 5 and 10 years was found to be 89%, 77% and 60%, respectively. The Markov chain predicted a life expectancy of 13.9 years. Central nervous system and renal involvement were poor prognostic factors. Proteinuria (> 0.5 g/day) caused a 50% reduction in life expectancy but increased disease activity at onset did not predispose to a poor outcome. CONCLUSION: The survival of patients with systemic lupus erythematosus continues to be poor. Central nervous system and renal disease indicate a poor outcome. Hence, new treatment strategies must be evolved to improve the survival of such patients.