Anti-C1q como Marcador de Nefritis Lúpica / Anti-C1q as Lupus Nephritis Marker

Introducción: Los autoanticuerpos anti-C1q han sido propuestos como un marcador útil en el lupus eritematoso sistémico por su asociación con la nefritis lúpica. Objetivo: Determinar la prevalencia de anti-C1q en pacientes con lupus eritematoso sistémico y otras enfermedades reumáticas para la evaluar la asociación con la nefropatía lúpica. Métodos: Se incluyeron 179 pacientes con lupus eritematoso sistémico y 82 con otras enfermedades reumáticas. La nefritis lúpica fue diagnosticada en 70 (39 por ciento) de los pacientes con lupus eritematoso sistémico. Los anticuerpos anti-C1q IgG se determinaron por ELISA. Las asociaciones se evaluaron por análisis de regresión logística. Resultados: La prevalencia de anti-C1q fue de 37 poe ciento (66/179) en los pacientes con lupus eritematoso sistémico y de 9 por ciento (7/82) en controles (OR = 6,3; IC 95 por ciento 2,8-14,1; p < 0,001). El anti-C1q fue asociado con proteinuria (OR = 2,6; IC 95 por ciento 1,2-6,0; p < 0,022); eritrosedimentación elevada (OR = 3,2; IC 95 por ciento 1,5-6,7; p < 0,003) y anti-DNAdc (OR = 3,9; IC 95 por ciento 1,7-9,1; p < 0,002). En el modelo de regresión logística ajustado para demografía y anti-DNAdc, aunque la OR del anti-C1q para la nefritis fue 2 veces más alta que en ausencia del anti-C1q, solo se aproximó a la significación estadística. La positividad simultánea de anti-C1q y anti-DNAdc estuvo asociada a la nefritis lúpica (OR = 4,3; IC 95 por ciento 1,9-9,5; p < 0,001). Conclusiones: El anti-C1q se presentó con mayor frecuencia en pacientes con lupus eritematoso sistémico que en los controles. El anti-C1q combinado con anti-DNAdc resultó fuertemente asociado a la nefritis lúpica(AU)

Introducción: Anti-C1q autoantibodies have been proposed as useful marker in systemic lupus erythematosus due to their association with lupus nephritis. Objective: To determine the prevalence of anti-C1q in patients with systemic lupus erythematosus and other rheumatic diseases to evaluate the association with lupus nephropathy. Methods: One hundred seventy-nine patients with systemic lupus erythematosus and 82 with other rheumatic diseases were included. Lupus nephritis was diagnosed in 70 (39percent) of patients with systemic lupus erythematosus. Anti-C1q IgG antibodies were determined by ELISA. Associations were evaluated by logistic regression analysis. Results: The prevalence of anti-C1q was 37percent (66/179) in patients with systemic lupus erythematosus and 9percent (7/82) in controls (OR = 6.3; 95percent CI 2.8-14). .1; p < 0.001). Anti-C1q was associated with proteinuria (OR = 2.6; 95percent CI 1.2-6.0; p < 0.022); elevated erythrocyte sedimentation rate (OR = 3.2; 95percent CI 1.5-6.7; p < 0.003) and anti-dsDNA (OR = 3.9; 95percent CI 1.7-9.1; p < 0.002). In the logistic regression model adjusted for demographics and anti-dsDNA, although the OR of anti-C1q for nephritis was 2-fold higher than in the absence of anti-C1q, it only approached statistical significance. Simultaneous positivity of anti-C1q and anti-dsDNA was associated with lupus nephritis (OR = 4.3; 95percent CI 1.9-9.5; p < 0.001). Conclusions: Anti-C1q occurred more frequently in patients with systemic lupus erythematosus than in controls. Anti-C1q combined with anti-dsDNA was strongly associated with lupus nephritis(AU)

Manifestaciones neurológicas en una paciente con lupus eritematoso sistémico / Neurological manifestations in a patient with systemic lupus erythematosus

El lupus eritematoso sistémico es una enfermedad inflamatoria sistémica cuyo proceso inflamatorio genera un importante número de manifestaciones clínicas articulares y extraarticulares. Entre las manifestaciones extraarticulares destaca la afectación neurológica, y el compromiso puede ser tanto a nivel del sistema nervioso central o periférico. El objetivo de la presente investigación es presentar el caso de una paciente de 56 años de edad, con diagnóstico de lupus eritematoso sistémico de 18 años de evolución quien presentó un cuadro crónico de cefalea, neuropatía periférica y miastenia gravis como manifestaciones neurológicas de la enfermedad. Con el tratamiento de ciclofosfamida y el aumento de la dosis de esteroide, en la actualidad la paciente se encuentra estable. El reporte de este caso hace hincapié en la importancia que revisten las manifestaciones neurológicas como parte de las manifestaciones extraarticulares de esta enfermedad, ya que este tipo de afección es determinante en el curso del lupus eritematoso sistémico y en la calidad de vida de los pacientes(AU)

Systemic lupus erythematosus is a systemic inflammatory disease whose inflammatory process generates a significant number of articular and extra-articular clinical manifestations. Within the extra-articular manifestations, neurological involvement stands out, among others, the commitment can be both at the level of the central or peripheral nervous system. The objective of the present investigation is to present the case of a 56-year-old patient, diagnosed with systemic lupus erythematosus of 18 years of evolution and who has presented chronic symptoms of headache, peripheral neuropathy and myasthenia gravis as neurological manifestations of the illness. The report of this case is considered important to publicize the importance of neurological manifestations as part of the extra-articular manifestations of this disease; this type of condition is decisive in the course of the disease and in the quality of life of patients(AU)

Clinical features of patients with Rhupus syndrome / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinical and serological features of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) overlap syndrome (Rhupus syndrome).@*METHODS@#We retrospectively reviewed the medical records of 21 patients with Rhupus syndrome who were hospitalized at Department of Rheumatology and Immunology, People's Hospital of Xinjiang Uygur Autonomous Region between January 2010 and January 2018. We compared the joint involvement, autoantibodies and clinical manifestations of Rhupus syndrome with 81 cases of RA-alone and 51 cases of SLE-alone.@*RESULTS@#In 21 patients with Rhupus syndrome, there are 3 males and 18 females. Compared with the SLE-alone group, the patients with Rhupus syndrome were older [(49.43±11.66) vs. (40.59±12.73), P=0.008]. The median age of the patients with Rhupus syndrome at RA onset was significantly younger than that of the RA-alone patients [(32.58±11.14) vs. (43.11±11.83), P=0.010]. Of the 21 patients with Rhupus syndrome, the initial diagnosis was RA in 57% (12/21), except 2 male patients, the other 10 patients with SLE manifestations were menopause, the mean age of amenorrhea or menopause was (44.30±5.33) (36-50) years. The mean interval between the onset of SLE and RA was 10.83 years. Two patients started with SLE manifestations. Moreover, both diseases simultaneously developed in 33.3% of the patients. Except one male patient, 3 patients were in menopause stage when RA and SLE appeared. The positive rate of specific antibody Rhupus syndrome was similar to that of RA. Renal damage was relatively rare in SLE related manifestations, but the incidence of interstitial lung disease was higher. There were no significant differences in the prevalence of complements C3 and C4, antinuclear antibody (ANA), anti-double-stranded DNA (anti-dsDNA), anti-SSA or anti-SSB antibody between the Rhupus syndrome and SLE-alone group.@*CONCLUSION@#Rhupus syndrome is an overlapping syndrome in which RA and SLE coexist. Most of the diseases occur in RA and the related manifestations of RA are more serious than those of SLE. The incidence of Rhupus syndrome may be related to the change of sex hormone levels.

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

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

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

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

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

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

Características clínico-epidemiológicas de los pacientes de la cohorte Lupus Paraguay (2013-2014) / Characteristics clinical-epidemiological of the patients of the Lupus Paraguay cohort (2013-2014)

El lupus eritematoso sistémico (LES) es una enfermedad autoinmune, de etiología desconocida que presenta manifestaciones clínicas de gravedad y curso impredecible. Se ha observado en varias cohortes que esta enfermedad tiene un comportamiento más agresivo en la población no caucásica debido a diferencias socioeconómicas, demográficas y genéticas. El objetivo del estudio es describir las características clínico-epidemiológicas de los pacientes con LES incluidos en la cohorte LUPUS-PY y la situación actual de las muestras custodiadas en el BIOBANCO IMID-PY. Estudio basado en la revisión de historias clínicas y en los cuestionarios clínico-epidemiológicos del día de la inclusión al registro LUPUS-PY. Se calcularon las frecuencias para las variables categóricas y las medias con su desvío estándar para las continuas. Se incluyeron de forma consecutiva, 83 pacientes con diagnóstico de LES (edad media: 34,4±10,0 años), el 86,7% era del sexo femenino, el 55% provenía de Asunción, el 49,1% tenía nivel educativo básico, el 45% percibía un ingreso mensual de un sueldo mínimo (aproximadamente 350 dólares americanos) o menos. El 46,8% no realizaba ningún tipo de actividad física. La media del tiempo trascurrido desde el inicio de los síntomas y el diagnóstico del LES fue de 9,3±13,7 años y el tiempo de evolución de la enfermedad hasta el ingreso al registro fue de 5,9±6,3. El 16% refirió antecedentes familiares de enfermedades autoinmunes. La primera manifestación de la enfermedad más descripta fue la articular (45%). Los pacientes con LES de nuestro centro tienen características tanto clínicas como epidemiológicas similares a las reportadas por otras cohortes(AU)

Clinical and epidemiologic characterization of patients with systemic lupus erythematosus admitted to an intensive care unit in Colombia

Abstract Objective: Describe the clinical and epidemiologic characteristics of patients with systemic lupus erythematosus (SLE) admitted to the intensive care unit (ICU). Methods: a retrospective study with medical records review of patients with systemic lupus erythematosus (SLE) admitted to the ICU between 2004 and 2015 were included. Qualitative variables were described using absolute and relative frequencies. For quantitative variables mean value and standard deviation (SD) or median value with the interquartile range (IQR) depending on data distribution. To compare groups, it was used the Student t-test or Mann Whitney U test as appropriate and Fisher's exact test. Results: 33 patients were included, with a total of 45 ICU admissions, 29 (87.9%) were females with a median age of 26 years. The median time of diagnosis of SLE was two years, (IQR 1.5-5). The most common SLE manifestation and comorbidity were renal disease and hypertension with 27 (81.8%) and 14 (42.4%) respectively. The main reason for admittance was lupus flare with 25 events (55.5%). Infection was the second cause of admission with 19 events (42.2%). The median stay time in the ICU was four days (IQR 2-7). LODS score was 6 (RIQ 5-8), and APACHE II score was 13 (RIQ 11-17.7). There were 29 infections (64.5%) of which 20 (69%) were hospital-acquired. Four (12.1%) patients died. Conclusion: Unlike most of the previously reported series, in this study SLE activity was the most common cause of admission in the ICU. A more aggressive disease and difficulties in the ambulatory setting could explain this behavior. Despite the higher percentage of lupus flares, there was lower mortality.

Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study

Abstract Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis. Methods: Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p < 0.004). Results: Panniculitis was observed in 6/847(0.7%) cSLE. Painful subcutaneous erythematosus and indurated nodules were observed in 6/6 panniculitis patients and painful subcutaneous plaques in 4/6. Generalized distribution was evidenced in 3/6 and localized in upper limbs in 2/6 and face in 1/6. Cutaneous hyperpigmentation and/or cutaneous atrophy occurred in 5/6. Histopathology features showed lobular panniculitis without vasculitis in 5/6(one of them had concomitant obliterative vasculopathy due to antiphospholipid syndrome) and panniculitis with vasculitis in 1/6. Comparison between cSLE with panniculitis and 60 cSLE without panniculitis with same disease duration [2.75(0-11.4) vs. 2.83(0-11.8) years,p = 0.297], showed higher frequencies of constitutional involvement (67% vs. 10%,p = 0.003) and leukopenia (67% vs. 7%,p = 0.002). Cutaneous atrophy and hyperpigmentation occurred in 83% of patients. Conclusions: Panniculitis is a rare skin manifestation of cSLE occurring in the first three years of disease with considerable sequelae. The majority of patients have concomitant mild lupus manifestations.

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

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

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

Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus / Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância

Abstract Objectives: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p < 0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p = 0.003), fever (32% vs. 56%, p = 0.020) and hepatomegaly (4% vs. 23%, p = 0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p > 0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.

Resumo Objetivos: Avaliar a vasculite digital (VD) clínica como uma manifestação inicial do lúpus eritematoso sistêmico de início na infância (LESi) em uma grande população. Métodos: Estudo transversal multicêntrico que incluiu 852 pacientes com LESi (critérios do ACR), acompanhados em dez centros de reumatologia pediátrica do Estado de São Paulo. Resultados: Observou-se VD em 25/852 (3%) pacientes com LESi. Diagnosticaram-se hemorragia periungueal em 12 (48%), infarto periungueal em sete (28%), úlcera de ponta de dígito em quatro (16%), nódulos dolorosos em um (4%) e gangrena em um (4%). Um desfecho ruim, com reabsorção digital, ocorreu em cinco (20%) pacientes. A comparação entre pacientes com e sem VD revelou maior frequência de erupção malar (80% vs. 53%, p = 0,008), erupção discoide (16% vs. 4%, p = 0,017), fotossensibilidade (76% vs. 45% p = 0,002) e outras vasculites cutâneas (80% vs. 19%, p < 0,0001), enquanto a frequência de características constitucionais totais (32% vs. 61%, p = 0,003), febre (32% vs. 56% p = 0,020) e hepatomegalia (4% vs. 23%, p = 0,026) foram menores nesses pacientes. A frequência do gênero feminino, o envolvimento grave de múltiplos órgãos, perfil de autoanticorpos e baixo complemento foram semelhantes nos dois grupos (p > 0,05). A mediana no Sledai-2 K, exclusive o descritor de VD, foi significativamente menor nos pacientes com VD em comparação com aqueles sem essa manifestação [10 (0 a 28) vs. 14 (0 a 58), p = 0,004]. Não foram observadas vasculite visceral nem morte nessa coorte de pacientes com LESi. A frequência de uso de ciclofosfamida (0% vs. 18%, p = 0,014) foi significativamente menor no grupo VD. Conclusão: Este grande estudo multicêntrico identificou a VD clínica como uma rara manifestação inicial do LESi ativo, associada a doença multissistêmica leve, apesar da ocorrência de reabsorção digital em alguns desses pacientes.

Familial autoimmunity and polyautoimmunity in 60 Brazilian Midwest patients with systemic sclerosis / Autoimunidade familial e poliautoimunidade em 60 pacientes portadores de esclerose sistêmica da região Centro-Oeste do Brasil

ABSTRACT Introduction: Systemic sclerosis (SSc) is a connective tissue disease of unknown etiology, characterized by a triad of vascular injury, autoimmunity and tissue fibrosis. It is known that a positive family history is the greatest risk factor already identified for the development of SSc in a given individual. Preliminary observation of a high prevalence of polyautoimmunity and of familial autoimmunity in SSc patients support the idea that different autoimmune phenotypes may share common susceptibility variants. Objectives: To describe the frequency of familial autoimmunity and polyautoimmunity in 60 SSc patients in the Midwest region of Brazil, as well as to report the main autoimmune diseases observed in this association of comorbidities. Methods: A cross-sectional study with recruitment of 60 consecutive patients selected at the Rheumatology Department, University Hospital, Medicine School, Federal University of Mato Grosso do Sul (FMUFMS), as well as interviews of their relatives during the period from February 2013 to March 2014. Results: A frequency of 43.3% of polyautoimmunity and of 51.7% of familial autoimmunity in SSc patients was found. Patients with the presence of polyautoimmunity and familial autoimmunity presented primarily the diffuse form of SSc, but this indicator did not reach statistical significance. The autoimmune diseases most frequently observed in polyautoimmunity patients were: Hashimoto's thyroiditis (53.8%), Sjögren's syndrome (38.5%), and inflammatory myopathy (11.5%). The main autoimmune diseases observed in SSc patients' relatives were: Hashimoto's thyroiditis (32.3%), rheumatoid arthritis (22.6%), and SLE (22.6%). The presence of more than one autoimmune disease in SSc patients did not correlate with disease severity or activity. Conclusions: From the high prevalence of coexisting autoimmune diseases found in SSc patients, we stress the importance of the concept of shared autoimmunity, in order to promote a continued vigilance and promptly diagnose other possible autoimmune disease in patients, or in their kin.

RESUMO Introdução: A esclerose sistêmica (ES) é uma enfermidade do tecido conjuntivo de etiologia desconhecida, caracterizada pela tríade de injúria vascular, autoimunidade e fibrose tecidual. Sabe-se que uma história familiar positiva representa o maior fator de risco já identificado para o desenvolvimento da ES em um determinado indivíduo. Observação prévia de alta prevalência de poliautoimunidade e de autoimunidade familiar em pacientes com ES reforça a ideia de que fenótipos autoimunes distintos podem dividir variantes comuns de susceptibilidade. Objetivos: Descrever a frequência de autoimunidade familiar e de poliautoimunidade em 60 pacientes com ES da região Centro-Oeste do Brasil, bem como relatar as principais doenças autoimunes observadas nessa associação de comorbidades. Métodos: Estudo transversal com recrutamento de 60 pacientes consecutivos, selecionados no Serviço de Reumatologia do Hospital Universitário da Faculdade de Medicina da Universidade Federal de Mato Grosso do Sul (FMUFMS), bem como entrevista de seus parentes, de fevereiro de 2013 a março de 2014. Resultados: Foi encontrada uma frequência de 43,3% de poliautoimunidade e de 51,7% de autoimunidade familiar nos pacientes com ES. Os pacientes com presença de poliautoimunidade e de autoimunidade familiar eram principalmente da forma difusa de ES, porém esse índice não atingiu significância estatística. As doenças autoimunes mais comumente observadas nos pacientes com poliautoimunidade foram: tireoidite de Hashimoto (53,8%), síndrome de Sjögren (38,5%) e miopatia inflamatória (11,5%). As principais doenças autoimunes observadas nos parentes dos pacientes com ES foram: tireoidite de Hashimoto (32,3%), artrite reumatoide (22,6%) e LES (22,6%). A presença de mais de uma enfermidade autoimune em pacientes com ES não se correlacionou com maior gravidade ou atividade da doença. Conclusões: A partir da alta prevalência encontrada de doenças autoimunes coexistentes em pacientes com ES, salientamos a importância do conceito de autoimunidade compartilhada, de forma a promover uma vigilância constante e diagnosticar prontamente uma possível outra doença autoimune nos pacientes ou em seus parentes.

Evaluación de estrés oxidativo en pacientes con Lupus Eritematoso Sistémico y su posible relación con la exposición ambiental a agroquímicos / Assessment of oxidative stress in patients with Systemic Lupus Erythematosus and its possible relationship with environmental exposure to agrochemicals

Los agroquímicos son un método efectivo para controlar especies perjudiciales a los intereses del hombre, pero aplicados indiscriminadamente provocan diversos impactos a nivel ambiental y en la salud. Uno de los mecanismos más importantes por el cual los agroquímicos se ponen en contacto directo con las poblaciones de zonas periurbanas es mediante la deriva. El Lupus Eritematoso Sistémico (LES) es una enfermedad autoinmune que posee un amplio rango de presentaciones clínicas y severidad. Si bien su etiología es desconocida, se la asocia con diversas causas ambientales dentro de las que podrían estar presentes los agroquímicos. El objetivo de este estudio fue evaluar, el posible daño oxidativo generado por exposición ambiental a mezclas de agroquímicos en pacientes con LES y personas sanas que residen en zonas rurales expuestas a mezclas de plaguicidas y contrastar los resultados con pacientes y personas sanas de zonas urbanas no expuestas. Se evaluaron 44 pacientes con diagnóstico de LES y 58 personas sanas (controles), se utilizaron como marcadores: catalasa (CAT), superóxido dismutasa (SOD), la relación glutation oxidado/reducido (GSH/GSSG) y lipoperoxidación (TBARS). Se encontraron diferencias estadísticamente signifcativas para TBARS y SOD al comparar los resultados del grupo LES urbano con su control y el grupo LES rural con el suyo (p<0,01 en ambos). Al realizar las comparaciones entre LES urbano y LES rural se halló un incremento en TBARS del 18,3% (p=0,014). Esta investigación demuestra la importancia de la utilización de biomarcadores de daño oxidativo en el seguimiento clínico de pacientes con LES, con el fn de establecer pautas de tratamiento adecuadas y considerar la relación entre la exposición ambiental a plaguicidas y el aumento de daño oxidativo en pacientes con LES.

The agrochemicals are an effective method to control harmful species of interests to humans, but when they are applied indiscriminately, they can cause environmental and health damage to people. Systemic Lupus Erythematosus (SLE) is an autoimmune disease that has a wide range of clinical manifestations and severity. While the etiology of SLE is unknown, it is associated with various environmental causes, being one possibility the exposure to agrochemicals. The aim of this study was to evaluate the possible oxidative damage generated by environmental exposure to agrochemical mixtures in SLE patients and healthy people living in rural areas and compare them with patients and healthy people living in urban, unexposed areas. Forty four patients diagnosed with SLE and 58 healthy individuals (controls) were evaluated, using the following markers: Catalase (CAT), superoxide dismutase (SOD), the oxidized/reduced glutathione ratio (GSH / GSSG) and lipid peroxidation (TBARS). Statistically signifcant differences were found in TBARS and SOD by comparing the results of the urban SLE group with its control and rural SLE group with its control (p <0.01 for both cases). When making comparisons between urban SLE group and the rural SLE group, an increase of 18.3% was found in TBARS (p = 0.014). This research demonstrates the importance of using biomarkers of oxidative stress in the clinical monitoring of patients with SLE, in order to establish guidelines for appropriate treatment and consider the relationship between environmental exposure to pesticides and increased oxidative damage in patients with SLE.

Mild and moderate Mannose Binding Lectin deficiency are associated with systemic lupus erythematosus and lupus nephritis in Brazilian patients / As deficiências leve e moderada de lectina ligadora de manose estão associadas ao lúpus eritematoso sistêmico e à nefrite lúpica em pacientes brasileiros

Abstract Objective The potential association of mannose binding lectin (MBL) deficiency and systemic lupus erythematosus (SLE) has been investigated in several studies, but results have been mixed. One explanation for the conflicting results could be differences in ethnic background of study subjects. In this study we investigated the association of MBL deficiency and SLE in a large cohort of Brazilian SLE patients and controls. Methods Serum MBL and Complement levels were determined for 286 Brazilian adult SLE patients and 301 healthy Brazilian adults as controls. MBL deficiency was classified as mild (<1000 and ≥500 µg/L), moderate (<500 and ≥100 µg/L) or severe (<100 µg/L). Results SLE patients presented higher frequency of mild and moderate MBL deficiency compared to controls. SLE patients with MBL deficiency presented higher frequency of lupus nephritis compared to those without MBL deficiency. MBL deficiency was not associated with any other clinical manifestation, use of immunosuppressant therapy, disease activity, disease severity serum or Complement levels. Conclusion This study shows that an association between MBL deficiency and SLE does exist in the Brazilian population. We also found an association between MBL deficiency and lupus nephritis. These findings support the hypothesis that MBL deficiency contributes to the development of SLE and lupus nephritis.

Resumo Objetivo Vários estudos já investigaram a potencial associação entre a deficiência de lectina de ligação a manose (LLM) e o lúpus eritematoso sistêmico (LES), mas os resultados obtidos são controversos. Uma explicação para esses resultados conflitantes poderia estar nas diferenças étnicas dos indivíduos estudados. Este estudo investigou a associação entre a deficiência de LLM e o LES em uma grande coorte de pacientes brasileiros com LES e controles. Métodos Determinaram-se os níveis séricos de LLM e complemento em 286 pacientes adultos brasileiros com LES e 301 adultos brasileiros saudáveis que atuaram como controles. A deficiência de LLM foi classificada como leve (< 1000 e ≥ 500 µg/L), moderada (< 500 e ≥ 100 µg/L) ou grave (< 100 µg/L). Resultados Os pacientes com LES apresentaram maior frequência de deficiências leve e moderada de LLM em relação aos controles. Os pacientes com LES com deficiência de LLM apresentaram maior frequência de nefrite lúpica em comparação com aqueles sem deficiência de LLM. A deficiência de LLM não esteve associada a qualquer outra manifestação clínica, uso de terapia imunossupressora, atividade da doença, gravidade da doença ou níveis séricos de complemento. Conclusão Este estudo mostra que há uma associação entre a deficiência de LLM e o LES na população brasileira. Encontrou-se também uma associação entre a deficiência de LLM e a nefrite lúpica. Esses resultados apoiam a hipótese de que a deficiência de LLM contribui para o desenvolvimento do LES e da nefrite lúpica.

IgA nephropathy in systemic lupus erythematosus patients: case report and literature review / Nefropatia por IgA em paciente portadora de lúpus eritematoso sistêmico: relato de caso e revisão de literatura

Abstract Systemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non‐lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1,280, nuclear fine speckled pattern, and anticardiolipin IgM and 280 U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non‐SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in the short and long term.

Resumo O lúpus eritematoso sistêmico (LES) é uma doença autoimune multissistêmica que tem como uma das manifestações mais marcantes a nefrite. Apesar de poder coexistir com outras doenças autoimunes e determinar a predisposição a diversas complicações infecciosas, o LES raramente é descrito em associação a nefropatias de etiologia não lúpica. Relatamos o caso da rara associação entre LES e nefropatia por IgA (NIgA) primária, a glomerulopatia primária mais frequente na população mundial. A paciente foi diagnosticada com LES pela ocorrência de eritema malar, alopecia, derrame pleural, proteinúria, pancitopenia, FAN 1:1.280 padrão nuclear pontilhado fino e anticardiolipina IgM 280 U/mL. A biópsia renal revelou hipercelularidade mesangial com depósitos isolados de IgA, compatível com NIgA primária. Foi tratada com antimalárico, prednisona e inibidor da enzima conversora de angiotensina e apresentou boa evolução. Por consistirem em doenças relativamente frequentes, a coexistência de LES e NIgA pode ser de fato um achado incomum por motivos desconhecidos ou uma condição subdiagnosticada. Este relato atenta para a importância da distinção entre a atividade de doença renal do LES e nefropatias não lúpicas, em especial a NIgA, definição que tem implicações importantes sobre o prognóstico renal e regimes terapêuticos a serem adotados em curto e longo prazo.

Hepatitis C virus antibodies in high risk juvenile onset systemic lupus erythematosus / Anticorpos contra o vírus da hepatite C em pacientes de alto risco com lúpus eritematoso sistêmico de início juvenil

Abstract Objective To evaluate the prevalence of hepatitis C virus (HCV) infection in high risk juvenile systemic lupus erythematosus (JSLE). Study design Forty low income JSLE patients (6M:34F; mean age 19 ± 4.4 yrs; mean disease duration 6 ± 3.2 yrs) were studied. Twenty healthy children and adolescents matched for social economical level were included as controls. Anti-HCV tests were performed using a third generation microparticle enzyme immunoassay. Inclusion criterion was low social economical level. Results The frequencies of anti-HCV antibody were low and comparable between JSLE and control group (2.5% vs. 0, p = 1.0). JSLE patients had significantly more risk factors for HCV infection compared to the control group, including immunosuppressive treatment (90% vs. 0, p < 0.0001), hospitalization (50% vs. 12.5%, p = 0.0006) and invasive procedures (47.5% vs. 12.5%, p = 0.001). Conclusions The observed low frequency of anti-HCV antibodies in high risk JSLE suggests that this virus does not seem to have a relevant role in the pathogenesis of this disease.

Resumo Objetivo Avaliar a prevalência de infecção pelo vírus da hepatite C (VHC) em pacientes de alto risco com lúpus eritematoso sistêmico de início juvenil (LESJ). Desenho do estudo Foram estudados 40 pacientes de baixa renda com LESJ (6 H: 34 M, com média de 19 ± 4,4 anos; duração média da doença de 6 ± 3,2 anos). Incluíram-se no grupo controle 20 crianças e adolescentes saudáveis pareados por nível socioeconômico. Fizeram-se testes anti-VHC com um ensaio imunoenzimático de micropartículas de terceira geração. O critério de inclusão foi o baixo nível socioeconômico. Resultados As frequências de anticorpos anti-VHC foram baixas e comparáveis entre os grupos LESJ e controle (2,5% versus 0, p = 1). Os pacientes com LESJ tinham significativamente mais fatores de risco para infecção por VHC em comparação com o grupo controle, incluindo tratamento imunossupressor (90% versus 0, p < 0,0001), internação (50% versus 12,5%, p = 0,0006) e procedimentos invasivos (47,5% versus 12,5%, p = 0,001). Conclusões A baixa frequência de anticorpos anti-VHC observada nos pacientes de alto risco com LESJ sugere que esse vírus não parece ter um papel relevante na patogênese dessa doença.

On lupus, vitamin D and leukopenia / Acerca de lúpus, vitamina D e leucopenia

Resumo Introdução A regulação imune está entre os efeitos não calcêmicos da vitamina D. Assim, essa vitamina pode influenciar em doenças autoimunes, como o lúpus eritematoso sistêmico (LES). Objetivos Estudar a prevalência da deficiência de vitamina D no LES e sua associação com o perfil clínico, sorológico e de tratamento, bem como com a atividade da doença. Métodos Mensuraram‐se os níveis séricos de OH‐vitamina D3 em 153 pacientes com LES e 85 controles. Os dados sobre o perfil clínico, sorológico e de tratamento de pacientes com lúpus foram obtidos por meio da revisão de prontuários. Simultaneamente à determinação da vitamina D, foi feito um hemograma e foi aplicado o Sledai (SLE disease activity índex [índice de atividade da doença no LES]). Resultados Os pacientes com LES tinham níveis mais baixos de vitamina D do que os controles (p = 0,03). Na análise univariada, a vitamina D sérica esteve associada à leucopenia (p = 0,02) e ao uso de ciclofosfamida (p = 0,007) e metotrexato (p = 0,03). Foi verificada uma correlação negativa com a dose de prednisona (p = 0,003). Não foi encontrada associação com a atividade da doença medida pelo Sledai (p = 0,88). Em um estudo de regressão múltipla, somente a leucopenia permaneceu como uma associação independente (B = 4,04; p = 0,02). Também foi encontrada correlação negativa do nível sérico de vitamina D com os granulócitos (p = 0,01), mas não com a contagem de linfócitos (p = 0,33). Conclusão Os pacientes com LES têm mais deficiência de vitamina D do que os controles. Essa deficiência não está associada com a atividade da doença, mas com a leucopenia (granulocitopenia).

Resumo Introdução A regulação imune está entre os efeitos não calcêmicos da vitamina D. Assim, essa vitamina pode influenciar em doenças autoimunes, como o lúpus eritematoso sistêmico (LES). Objetivos Estudar a prevalência da deficiência de vitamina D no LES e sua associação com o perfil clínico, sorológico e de tratamento, bem como com a atividade da doença. Métodos Mensuraram‐se os níveis séricos de OH‐vitamina D3 em 153 pacientes com LES e 85 controles. Os dados sobre o perfil clínico, sorológico e de tratamento de pacientes com lúpus foram obtidos por meio da revisão de prontuários. Simultaneamente à determinação da vitamina D, foi feito um hemograma e foi aplicado o Sledai (SLE disease activity índex [índice de atividade da doença no LES]). Resultados Os pacientes com LES tinham níveis mais baixos de vitamina D do que os controles (p = 0,03). Na análise univariada, a vitamina D sérica esteve associada à leucopenia (p = 0,02) e ao uso de ciclofosfamida (p = 0,007) e metotrexato (p = 0,03). Foi verificada uma correlação negativa com a dose de prednisona (p = 0,003). Não foi encontrada associação com a atividade da doença medida pelo Sledai (p = 0,88). Em um estudo de regressão múltipla, somente a leucopenia permaneceu como uma associação independente (B = 4,04; p = 0,02). Também foi encontrada correlação negativa do nível sérico de vitamina D com os granulócitos (p = 0,01), mas não com a contagem de linfócitos (p = 0,33). Conclusão Os pacientes com LES têm mais deficiência de vitamina D do que os controles. Essa deficiência não está associada com a atividade da doença, mas com a leucopenia (granulocitopenia).

Periodontitis and systemic lupus erythematosus / Doença periodontal e lúpus eritematoso sistêmico

ABSTRACT A large number of studies have shown a potential association between periodontal and autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus (SLE). Similar mechanisms of tissue destruction concerning periodontitis and other autoimmune diseases have stimulated the study of a possible relationship between these conditions. This study aims to review the literature about this potential association and their different pathogenic mechanisms. Considering that periodontal disease is a disease characterized by inflammation influenced by infectious factors, such as SLE, it is plausible to suggest that SLE would influence periodontal disease and vice-versa. However, this issue is not yet fully elucidated and several mechanisms have been proposed to explain this association, as deregulation mainly in innate immune system, with action of phagocytic cells and proinflammatory cytokines such as IL-1β and IL-18 in both conditions’ pathogenesis, leading to tissue destruction. However, studies assessing the relationship between these diseases are scarce, and more studies focused on common immunological mechanisms should be conducted to further understanding.

RESUMO Um grande número de estudos tem mostrado uma potencial associação entre doenças periodontais e doenças autoimunes, como artrite reumatoide e lúpus eritematoso sistêmico (LES). Os mecanismos de destruição tecidual semelhantes entre a periodontite e as demais doenças autoimunes têm estimulado o estudo de possíveis relações entre essas condições. O presente estudo tem como objetivo revisar a literatura acerca dessa potencial associação e dos seus diferentes mecanismos patogênicos. Considerando-se a doença periodontal uma doença de caráter inflamatório que sofre influência de fatores infecciosos, assim como o LES, é plausível sugerir que o LES influenciaria sua progressão, assim como a periodontite influenciaria a progressão do LES. Entretanto, essa questão ainda não é totalmente elucidada e vários mecanismos têm sido propostos para explicar tal associação, como desregulações, principalmente no sistema imune inato, com ações de células fagocíticas e de citocinas pró-inflamatórias, como IL-1β e IL-18, na patogênese de ambas as condições, o que contribui para a destruição tecidual. Existem, contudo, poucos estudos na literatura que avaliam a relação entre essas doenças e mais trabalhos focados nos mecanismos imunológicos comuns a ambas as condições devem ser feitos para um maior entendimento.

Infections and systemic lupus erythematosus / Infecções e lúpus eritematoso sistêmico

ABSTRACT Objective To determine the incidence of infections in a population of systemic lupus erythematosus individuals and the characteristics of infections regarding original site, as well as to study the possible associations between infections and treatment. Methods An analytical retrospective study using data from medical charts of systemic lupus erythematosus patients from a single university hospital. A total of 144 patients followed up for five years were included. Data collected comprised age of patients and age at onset of lupus, sex and ethnicity, disease duration before the study period, medications, cumulative dose of prednisone, occurrence of infections and their original site. Results The most frequent infections were urinary tract infections (correlated to use of prednisone − p<0.0001 and cyclophosphamide − p=0.045), upper airways infections (correlated to use of prednisone − p=0.0004, mycophenolate mofetil − p=0.0005, and cyclosporine − p=0.025), and pneumonia (associated to prednisone − p=0.017). Conclusion Prednisone was the drug more often associated with presence of infections, pointing to the need for a more judicious management of this drug.

RESUMO Objetivo Determinar a incidência de infecções em uma população com lúpus eritematoso sistêmico e a caracterização das mesmas quanto aos locais de origem, assim como estudar possíveis associações das infecções com a terapêutica utilizada. Métodos Estudo retrospectivo analítico utilizando dados de prontuários de indivíduos com lúpus eritematoso sistêmico de um único hospital universitário. Foram investigados 144 pacientes acompanhados pelo período de 5 anos. Os dados coletados incluíam idade do paciente e idade de início do lúpus, sexo e etnia, tempo de duração da doença antes do período do estudo, medicamentos administrados, dose cumulativa de prednisona, ocorrência de infecções e seu local de origem. Resultados As infecções mais observadas foram as do trato urinário (correlacionadas com o uso de prednisona − p<0,0001, e ciclofosfamida − p=0,045), infecções das vias aéreas superiores (correlacionadas com o uso de prednisona − p=0,0004, micofenolato de mofetila − p=0,0005 e ciclosporina − p=0,025) e pneumonia (correlacionadas ao uso de prednisona − p=0,017). Conclusão A prednisona foi o medicamento mais associado com a presença de infecções, apontando para a necessidade de um manejo mais judicioso desse medicamento.

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.

Acute Longitudinal Myelitis As The First Presentation in Child with Systemic Lupus Erythematosus Concurrent with Positive Antiphospholipid Antibody.

Systemic lupus erythematosus (SLE) is a multi-system auto-immune disorder that is characterized by widespread immune deregulation, formation of auto–antibodies, and immune complexes, resulting in infl ammation and potential damage to variety of organs. 25-95% it is complicated by neurological or neuropsychiatric symptoms, which is referred to as neuropsychiatric SLE (NPSLE). NPSLE contain both central and peripheral nervous systems, which includes transverse myelitis. We report our experience of concurrent manifestation of transverse myelitis as an initial presentation of SLE, which suggests the common immune-mediated mechanisms of diseases. We here report the case of a 7-year-old girl with SLE who fi rst presented with features of TM. Th e patient developed ascending weakness starting from low extremities, experienced diffi culty in voiding. An initial diagnosis of TM was made on the basis of clinical fi ndings and MRI spine, which displayed T2 weighted high signal intensities at thoracic level. She partially respond to intravenous immunoglobulin therapy, and serological analysis revealed the presence of anti-dsDNA, anti nuclear antibody with decreased level of complements. Th e diagnosis was revised to acute transverse myelitis resulting from SLE. Additional methylprednisolone pulse therapy led to rapid clinical improvement. Th is was followed by oral prednisolone and cyclophosphamide pulse therapy. Th e crossreactivity of auto-antibodies and increased susceptibility to infection owing to immunologic changes associated with lupus may form the basis of the association. Systemic Lupus Erytheromyitis should consider as an etiology of transverse myelitis. Aggressive treatment may alter the course and lead to a better outcome.