Tratamiento con rituximab en 2 pacientes con lupus eritematoso sistémico pediátrico

Introducción . El LES pediátrico (LESp) representa el 15% de todos los pacientes con LES. La afectación renal y neuropsiquiátrica es más agresivo en el LESp, siendo la afectación de un solo órgano la forma clínica de aparición más común. Formas de presentación como infarto cerebral y serositis son manifestaciones poco frecuentes en el LESp. El tratamiento de un LESp no difiere al de las formas adultas y el arsenal terapéutico es el mismo. El rituximab (RTX) es un agente biológico utilizado a nivel mundial en LES con excelentes resultados, sin embargo, aún no existe consenso sobre su eficacia real en LESp. Objetivos . Presentación de 2 casos de LESp con infarto cerebral y serositis como forma de presentación, que no respondieron a la terapia convencional pero sí a RTX. Métodos . Reporte de caso, con descripción del cuadro clínico, método diagnóstico y forma de tratamiento. Resultados. Primer caso: mujer de 16 años que consulta por cefalea progresiva con crisis tónico-clónica. La tomografía cerebral mostró un infarto cerebral frontoparietal izquierdo. En el examen físico se encontró livedo reticularis en miembros inferiores, dolor articular, caída del cabello y úlceras orales. Las pruebas revelaron anemia normocítica, trombocitopenia, disminución del complemento, 1/320 ANA con patrón homogéneo, 3.200 mg de proteína en muestra de orina de 24 horas y anticuerpos antifosfolipídicos negativos. Se realizó diagnóstico de LESp con compromiso renal, neurológico y hematológico, decidiendo uso de metilprednisolona 1 g EV diario durante 3 días, para luego pasar a ciclofosfamida 1 g EV mensual por 6 meses. Después de 3 meses persisten proteinuria, fatiga y artralgias. Por este motivo, se decidió utilizar rituximab a una dosis de 375 mg / m2 en días 1 y 15 cada 6 meses. Tras 4 infusiones, la proteinuria desapareció, así como las artralgias y malestar general. Actualmente mantiene scores SLEDAI-2K en remisión, con dosis bajas de prednisona. Segundo caso: niño de 10 años, presentó dolor abdominal difuso con distensión asociada de inicio más o menos abrupto. Se agregó cansancio, dificultad para respirar y palpitaciones. Una radiografía simple de abdomen no mostró niveles hidroaéreos, pero la placa torácica demostró derrame pleural bilateral con agrandamiento de la silueta cardíaca. Un ecocardiograma y una ecografía abdominal reveló derrame pericárdico y ascitis respectivamente. Al examen físico se observó palidez general, edema translúcido de miembros inferiores, roce pericárdico y disminución del soplo vesicular en ambas bases pulmonares. Las pruebas de laboratorio mostraron leucopenia, linfopenia, anemia normocítica, reactantes de fase aguda elevados, ANA 1/560, anti-ADN 280 U / mL, complemento disminuido, transaminasas elevadas, urea y creatinina normales. Se diagnosticó LESp y se pulsó con metilprednisolona 30 mg / kg / dosis durante 4 días, para luego pasar a micofenolato 600 mg / m2 diarios. Inicialmente hubo mejoría, pero después de 2 meses reapareció la serositis inicial. Se decidió usar rituximab 375 mg / m2. Después de la segunda infusión la serositis desapareció, normalizándose valores de hemograma, complemento y transaminasas. Actualmente se encuentra en remisión, con dosis bajas de prednisona. Conclusión . Los casos presentados debutaron con infarto cerebral y serositis, mostraron ANA elevados títulos y disminución del complemento. Ambos casos mejoraron con rituximab tras el fracaso a ciclofosfamida y micofenolato. Se obtuvo el consentimiento informado de los padres y los pacientes.

Introduction . Pediatric systemic lupus erythematosus (pSLE) represents 15% of all SLE patients. Renal and neuropsychiatric involvement are more aggressive in pSLE, and single organ involvement is the most commonly found clinical form. Conditions such as cerebral infarction and serositis are unusual manifestation of pSLE. Therapy for pSLE is not different from that for the adult forms, and the therapy armamentarium is the same. Rituximab (RTX) is a worldwide used biological for SLE, with excellent results; however, there is still no consensus with respect to is real efficacy in pSLE. Objectives . Presentation of two pSLE cases with cerebral infarction and serositis as main characteristics, who did not respond to conventional therapy, but who did respond to RTX. Methods . Case report, describing the clinical presentation, diagnostic methods, and therapy approach used. Results . First case: This is a sixteen-year-old girl who was brought because of progressing headache and a tonic-clonic crisis. The brain CT scan showed a left frontoparietal cerebral infarction. Physical examination revealed livedo reticularis in both legs, joint pain, hair loss, and mouth ulcers. Laboratory tests revealed normocytic anemia, thrombocytopenia, reduced complement, 1/320 ANA with a homogeneous pattern, 3.200 mg 24-hour proteinuria, and negative anti-phospholipidic antibodies. A pSLE diagnosis was made, with renal, neurologic, and hematologic involvement, so it was decided to use methylprednisolone, 1 gram IV per day for three days, and then switch to cyclophosphamide 1 g IV per month for 6 months. After three months, proteinuria, fatigue, and arthralgia persisted. For this reason, it was decided to administer rituximab, 375 mg/m2 in days 1 and 15, every six months. After four infusions, proteinuria, joint pain and malaise all disappeared. Nowadays this patient maintains SLEDAI-K scores in remission, and she is also receiving low-dose prednisone. Second case: This is a ten-year-old boy, who presented with abrupt diffuse abdominal pain associated with (abdominal) distention. Other manifestations were tiredness, shortness of breath, and palpitations. A plain abdomen X-ray film did not show hydro-aerial levels, but the chest X-ray film showed bilateral pleural effusion, and enlarged cardiac silhouette. Cardiac ultrasonography and abdominal ultrasonography revealed pericardial effusion and ascites, respectively. Findings in physical examination showed pallor translucid edema of the legs, pericardial throbbing, and reduced respiratory sounds in both pulmonary bases. Laboratory tests revealed leukopenia, lymphopenia, normocytic anemia, elevated acute phase reactants, ANA 1/560, anti-DNA 280 U/mL, reduced complement, elevated transaminases, and normal urea and creatinine. pSLE was diagnosed, and therapy instituted was methylprednisolone 30 mg/Kg/dose for 4 days, then it was switched to mycophenolate 600 mg/m2 per day. There was improvement initially, but after two months, serositis reappeared. Then it was decided to start rituximab 375 mg/m2. After the second infusion, serositis disappeared, and CBC, complement, and transaminase values returned to normal. Nowadays the patient is in remission, and he is receiving low-dose prednisone. Conclusión . Both presented cases featured cerebral infarction and serositis. They also showed high ANA titers and reduced complement. Both patients improved their condition with rituximab after failure with cyclophosphamide and mycophenolate. Informed consent from both parents and patients was obtained.

Evaluation of serum microRNA-30e-5p expression in systemic lupus erythematosus and its association with clinical manifestations: A cross-sectional study / Evaluación de la expresión del microARN-30e-5p sérico en el lupus eritematoso sistémico y su asociación con las manifestaciones clínicas: un estudio transversal

ABSTRACT Background: MicroRNAs (miRNAs) are non-coding RNAs that regulate gene expression post-transcriptionally. Accumulating evidence indicates that the miR-30 family takes part in the development of multiple tissues and organs, and is a potential contributor to various dis eases, including autoimmune disorders such as systemic lupus erythematosus (SLE). The aim of this study was to evaluate the expression of miR-30e-5p, a member of the miR-30 fam ily, and investigate its potential relationship to clinical characteristics and possible disease activity in an Egyptian SLE cohort. Methods: Serum samples from 40 SLE patients and 37 age and gender matched healthy sub jects were tested for miR-30e-5p expression level using the Taqman quantitative reverse transcription-polymerase chain reaction. Analysis was performed using the 2 - AACT method. Results: The mean age of the patients was 28.7 ± 7.9 years, with a mean disease duration of 6.4 ±5.3 years. The median fold change in serum miR-30e-5p among our SLE cohort was significantly higher 1.748 (0.223-20.485) compared to the control group 0.877 (0.058-3.522) (P = 0.02). Receiver operating characteristic curve analysis revealed that miR-30e-5p expres sion level can discriminate SLE patients from controls at a cut-off value >1.06 with the area under the curve (AUC) = 0.676 (95% CI: 0.559-0.794, P = 0.02), with 64.3% sensitivity and 61.5% specificity. There was no correlation between any of the demographic features, clinical manifestations (apart from serositis, P = 0.013) or disease activity and miR-30e-5p levels. Conclusion: Our study demonstrated elevated miR-30e-5p expression levels in serum sam ples of SLE patients. Apart from serositis, it was not associated with any other disease characteristics.

RESUMEN Antecedentes: Los microARN (miRNA) son ARN no codificantes que regulan la expresión de los genes después de la transcripción. Las pruebas acumuladas indican que la familia de miR-30 participa en el desarrollo de múltiples tejidos y órganos, y es un posible contribuyente a diversas enfermedades, incluidos los trastornos autoinmunes como el lupus eritematoso sistémico (LES). El objetivo de este estudio fue evaluar la expresión del miR-30e-5p, un miembro de la familia miR-30, e investigar su posible relación con las características clínicas y la posible actividad de la enfermedad en una cohorte egipcia de LES. Métodos: Se analizaron muestras de suero de 40 pacientes con LES y 37 sujetos sanos de edad y sexo similares para determinar el nivel de expresión de miR-30e-5p, utilizando la reacción en cadena de la polimerasa de transcripción inversa cuantitativa Taqman. El análisis se llevó a cabo empleando el método 2-AACT. Los resultados: La edad media de los pacientes fue de 28,7 ± 7,9 años, mientras que la duración media de la enfermedad fue de 6,4 ± 5,3 años. La mediana del cambio de pliegue del suero miR-30e-5p entre nuestra cohorte de LES fue significativamente mayor, 1,748 (0,223-20,485), en comparación con el grupo de control, 0,877 (0,058-3,522) (p = 0,02). El análisis de la curva característica de funcionamiento del receptor reveló que el nivel de expresión del miR-30e-5p puede discriminar a los pacientes con LES de los controles en un valor de corte > 1,06, con el área bajo la curva (AUC) = 0,676 (IC del 95%: 0,559-0,794; p = 0,02), una sensibilidad del 64,3% y una especificidad del 61,5%. No hubo asociación entre ninguna de las características demográficas, manifestaciones clínicas (aparte de la serositis, p = 0,013) o actividad de la enfermedad y los niveles de miR-30e-5p. Conclusión: Nuestro estudio demostró niveles elevados de expresión de miR-30e-5p en mues tras de suero de pacientes con LES. Aparte de la serositis, no se asoció con ninguna otra característica de la enfermedad.

The correlation analysis between the neutrophil-lymphocyte ratio and systemic lupus erythematosus viscera involvement and disease activity / 中华肾脏病杂志

Objective To investigate the correlation between neutrophil-lymphocyte ratio (NLR) and disease activity of systemic lupus erythematosus (SLE),and the changes of NLR in different organ involvement of SLE patients.Methods A total of 155 SLE patients and 135 healthy controls from the Rheumatology Department of Xiangya Hospital were enrolled in this study from 2010 to 2018.Patients with SLE were divided into lupus nephritis group (LN group) and non-lupus nephritis group (non-LN group),serositis group and non-serositis group,according to whether they had kidney involvement or serositis.According to the SLE disease activity index 2000(SLEDAI-2000),the patients were divided into mild to moderate disease activity group (SLEDAI score ＜ 15) and severe disease activity group (SLEDAI score≥ 15).The NLR values of the above groups were compared.Spearman's correlation analysis was used to analyze the correlation between NLR and SLE patients' laboratory indexes.Multiple linear regression model was used to analyze the relationship between NLR and SLE disease activity.Receiver operating characteristic curve (ROC) was used to evaluate the value of NLR in SLE diagnosis and activity assessment.Results (1)The NLR value of SLE patients was significantly higher than that of healthy control group,and the difference was statistically significant (P ＜ 0.01).(2)The NLR value of SLE patients in the LN group was higher than that in the non-LN group,and the NLR value of SLE patients with serositis was higher than that in the group without serositis,with statistically significant differences (both P ＜ 0.05).(3)The NLR value of SLE patients in the severe disease activity group was higher than that in the mild and moderate disease activity group,and the difference was statistically significant (P ＜ 0.01).(4)NLR of SLE patients was positively correlated with CRP (rs=0.188,P=0.019),SLEDAI score (rs=0.264,P=0.001),and negatively correlated with total serum protein (rs=-0.250,P=0.002) and serum albumin (rs=-0.329,P ＜ 0.001),respectively.(5) Multiple linear regression showed that NLR was independently associated with SLE disease activity (B=0.351,95％CI 0.012-0.690,t=2.047,P=0.042).(6) According to ROC curve,the optimal cut-off value of NLR for SLE diagnosis was 2.17 (sensitivity 60.0％,specificity 83.1％,AUC=0.744),and the best cut-off value for predicting the activity of severe disease activity in SLE patients was 3.28 (sensitivity 58.5％,specificity 78.1％,AUC=0.700).Conclusion NLR is closely related to renal involvement,serositis and disease activity in SLE patients,which indicates that NLR,as a new inflammatory indicator,is of great significance for the assessment of SLE disease activity and organ involvement.

Doença sistêmica relacionada à IgG4 com linfopenia: relato de caso e breve revisão de literatura / IgG4-related systemic disease associated with lymphopenia: case report and brief literature review

A doença sistêmica relacionada à IgG4 é uma doença emergente, recentemente descrita, caracterizada clinicamente por aumento parcial ou total de um órgão, e, por isso, com amplo espectro de manifestações clínicas. Esta é uma doença sistêmica fibroinflamatória, patologicamente provocada pela infiltração de plasmoblastos IgG4 positivos que levam à inflamação eosinofílica do tecido e, consequentemente, fibrose estoriforme. Quando o diagnóstico é precoce, a melhora clínica e resposta sustentada com corticosteroides sistêmicos é impressionante. O diagnóstico é baseado em critérios patológicos, mas, recentemente, alguns trabalhos têm descrito que plasmoblastos no soro podem servir como um fator independente para auxiliar no diagnóstico da doença. Este artigo descreve uma apresentação atípica da doença relacionada à IgG4, em um paciente linfopênico com medição inconclusiva de plasmoblastos no soro.

IgG4-related systemic disease is a recently described, emerging condition, clinically characterized by partial or total enlargement of an organ, with a broad spectrum of clinical manifestations. It is a systemic fibroinflammatory condition caused by the infiltration of IgG4-positive plasmablasts that lead to eosinophilic inflammation of tissues and consequently storiform fibrosis. When diagnosis is early, clinical improvement and maintained response achieved with systemic corticosteroids is impressive. Diagnosis is based on pathological criteria, but recent papers have described that serum plasmablasts may serve as an independent factor to aid in diagnosis. This paper describes an atypical presentation of IgG4-related disease in a lymphopenic patient with inconclusive serum plasmablast measurement.

Polisserosite de Concato: uma doença rara ou pouco conhecida? / Concato's disease: a rare or a little known condition?

Polisserosite consiste na inflamação das serosas (pericárdio, pleurae peritônio) com efusão. Na polisserosite de Concato, além das manifestações inflamatórias das serosas, ocorre pericardite com espessamento dos folhetos do pericárdio, podendo levar a um quadro de pericardite constritiva. Justifica-se este estudo ao considerarmos que a etiologia das serosites são muitas; por isso, há necessidade de melhor estudá-las e difundi-las no meio acadêmico. Nesse sentido, propomo-nos a rever a literatura, por meio de levantamento bibliográfico, de pesquisa em rede e literatura atualizada sobre o tema abordado. Deste modo, pretendemos melhor elucidar a etiopatogenia da doença de Concato, dada a importância do diagnóstico precoce, combinado ao tratamento adequado, na prevenção de consequências indesejáveis e fatais. O principal objetivo desta revisão é meramente acadêmico, pois há um desconhecimento muito grande desta forma de apresentação de tal doença no meio médico. Acreditamos que, com este estudo de revisão, poderemos contribuir para a conscientização e efetivação do diagnóstico precoce da patologia estudada, evitando complicações e, assim, promover: saúde, multiplicação e difusão do conhecimento adquirido.

Polyserositis is the inflammation of serous membranes (pericardium, pleura and peritoneum) with effusion. In Concato’s disease, in addition to chronic inflammatory manifestations of serous membranes, there occurs pericarditis with thickening of pericardium leasflets, which may lead to a picture of constrictive pericarditis. This study is justified by the multiple etiological factors that may lead to serosites, and the need to further study and publish study results. In this sense, a review was proposed by making a literature survey using network research and current literature available on the topic for the purpose of elucidating the etiopathogenesis of Concato’s disease, given the importance of an early diagnosis, in association with the appropriate treatment, in the prevention of undesirable or even fatal consequences. The aim of this review is academic, inasmuch as there is lack of medical knowledge on the manifestations of this condition. By doing this, we aim to contribute to developing an awareness of the importance of an early diagnosis of this pathology, thus avoiding complications and promoting health and the propagation of acquired knowledge.

Taponamiento cardiaco por pericarditis lúpica: reporte de dos casos / Cardiac tamponade due to lupic pericarditis: report of two cases

La serositis es uno de los hallazgos clínicos en pacientes con lupus eritematoso sistémico.Cuando se presenta a nivel de pericardio, generalmente causa derrames pericárdicos cró-nicos que no generan inestabilidad hemodinámica en el paciente. Presentamos 2 casosclínicos de mujeres con lupus con derrame pericárdico que lleva a taponamiento cardiaco,presentación inusual de esta manifestación clínica del lupus eritematoso sistémico...

Serositis is one of the clinical findings in patients with systemic lupus erythematosus. Whenis present generally causes chronic pericardial effusions and not generate hemodynamicinstability in the patient. We present two cases of women with lupus with pericardial effusionleading to cardiac tamponade, unusual presentation of this clinical manifestation insystemic lupus erythematosus...

Ciento quince pacientes con lupus eritematososistémico: características clínicas e inmunológicas / A series of 115 patients with systemic lupus erythematosus: Clinical and immunological characteristics

Introducción: El lupus eritematoso sistémico es una enfermedad autoinmune, multisistémicade etiología desconocida, que afecta principalmente a mujeres en edad reproductiva. Dentrode las manifestaciones más frecuentes se encuentran los compromisos renal e inmunoló-gico; el primero es el responsable de gran parte de la morbimortalidad de los pacientes y elsegundo está fuertemente asociado a múltiples manifestaciones clínicas.Objetivo: Analizar la prevalencia y características de las principales manifestaciones clínicase inmunológicas de 115 pacientes con diagnóstico de lupus eritematoso sistémico del HospitalUniversitario San Vicente Fundación y establecer la asociación entre los anticuerposespecíficos y el compromiso de órgano.Materiales y métodos: Los pacientes fueron vistos por el Grupo de Reumatología de la Universidadde Antioquia entre 2008 y 2012. Los datos clínicos e inmunológicos se recolectaron delarchivo o sistema electrónico de historias clínicas del hospital, en un formato previamenteprotocolizado.Resultados y conclusiones: Similar a lo informado por otros estudios, se encontró asociaciónentre hipocomplementemia y compromiso renal, y mayor alteración de las pruebas defunción renal como creatinina, nitrógeno ureico en sangre, depuración de creatinina y proteinuriaen 24h. La frecuencia de anticuerpos anti-ADN de doble cadena fue del 79,1% y estosse encontraron asociados con compromiso renal y puntuaciones más altas de ®SystemicLupus Erythematosus Disease Activity Index¼. Se observó también asociación entre anticuerposanti-Sm con serositis y lupus discoide...

IntroductionSystemic lupus erythematosus is an autoimmune disease. The etiology is unknown, and it primarily affects women of reproductive age. Among the most common manifestations are the renal and immune system involvement; the first one is responsible for the majority of the morbidity and mortality of patients, and the second strongly associated with multiple clinical manifestations.ObjectiveTo analyze the prevalence and characteristics of the main clinical and immunological manifestations of 115 patients diagnosed with systemic lupus erythematosus in the Hospital Universitario San Vicente Fundación, and to determine the association between autoantibodies and organ involvement.Materials and methodsPatients were seen by the Rheumatology Group, University of Antioquia, between 2008 and 2012. Clinical and immunological data were collected from the files or electronic medical records using a previously designed format.Results and conclusionsThe study found an association between hypocomplementemia and renal involvement, similar to other studies. Nephritis was found in patients with active lupus, as well as a greater impairment of renal function tests such as, creatinine, blood urea nitrogen, creatinine clearance, and proteinuria. The percentage of patients with anti-dsDNA was 79.1%, and these antibodies were associated with renal involvement, as well as higher Systemic Lupus Erythematosus Disease Activity Index scores; anti-Sm antibodies was associated with serositis and discoid lupus...

Hemólisis, exantema, serositis. Manifestaciones extrapulmonares del Mycoplasma pneumoniae: Reporte de un caso / Hemolysis, serositis and exanthema induced by Mycoplasma pneumoniae infection: Report of one case

Mycoplasma infections have extrapulmonary manifestations that may be associated with respiratory symptoms and may have skin, heart, gastrointestinal, rheumatologic, neurologic, hematologic involvement. Cold agglutinin mediated autoimmune hemolytic anemia is the most common hematological manifestation. We report a 27-year-old woman infected with Mycoplasma pneumoniae, who presented respiratory involvement with pneumonia, exanthema, serositis and acute hemolytic anemia that required transfusion. The key for the diagnosis were the extrapulmonary manifestations associated with respiratory involvement after five days of hospitalization.

Lupus eritematoso sistémico en el anciano: una presentación atípica de una enfermedad común / Systemic lupus erythematosus in the elderly: An atypical presentation of a common disease

El lupus eritematoso sistémico (LES) del anciano, también llamado lupus de aparición tardía,es una enfermedad autoinmune que aparece después de los 50-60 años, con un curso clínicoy manifestaciones clínicas que difieren del LES clásico, cuya prevalencia es en personas másjóvenes, predominantemente mujeres. Se presenta, en este artículo, el caso de un pacientemasculino de 72 años con cuadro clínico de un mes de evolución de dolor en hemitóraxderecho, tipo pleurítico asociado a disnea, además de la presencia de lesiones eritematosasy descamativas en región malar y zona de exposición solar en tórax. Se descartó origeninfeccioso y neoplásico mediante imágenes diagnósticas y laboratorios y, posteriormente,se realiza perfil inmunológico que reporta ANAS positivo, Anti-DNA positivo y complementoconsumido, con evidencia de derrame pleural derecho masivo recidivante hasta la fecha.

Systemic lupus erythematosus (SLE) in the elderly, also called late-onset lupus, is an autoimmune disease that appears after 50-60 years old, with a clinical course and clinical manifestations that differ from classic SLE, with a prevalence predominantly in younger women. In this article a case of a 72 year-old male patient who, for one month, had clinical symptoms of right chest pain, associated with dyspnea and the presence of erythematous and scaly lesions on the malar area and sun exposure in the thorax. The possibilities of infectious or neoplastic origin were dismissed using diagnostic images and laboratory tests. An immunological profile was subsequently performed, reporting positive ANAS and Anti-DNA, positive, and consumed complement, with evidence of recurrent massive right pleural effusion to date.

A Case of Rheumatoid Pneumoconiosis Presenting with Pleuritis and Pericarditis / 대한내과학회지

Caplan's syndrome is characterized by multiple small distinct nodules with progressive massive fibrosis and rheumatic arthritis in pneumoconiosis. Although pleural effusions occur infrequently as an extra-articular manifestation, pleuritis can develop without joint involvement in patients with rheumatoid arthritis. We treated an 81-year-old man who had been diagnosed with silicosis with progressive massive fibrosis. He suffered from progressive dyspnea, and chest computed tomography (CT) and echocardiography revealed pleural and pericardial effusions. We speculated that the multiple serositis was related to a rheumatic disorder because the rheumatic factor was elevated in both the pleural and pericardial effusions. After corticosteroid treatment, the serositis improved. We suggest that this case is an atypical pattern of Caplan's syndrome presenting as serositis without arthritis. Rheumatoid serositis should be considered as the cause of pleural or pericardial effusions in patients with pneumoconiosis.

Ascite por peritonite lúpica: uma forma rara de início do lúpus eritematoso sistêmico / Ascites due to lupus peritonitis: a rare form of onset of systemic lupus erythematosus

O lúpus eritematoso sistêmico (LES) frequentemente cursa com serosites. Cerca de 16 por cento dos pacientes lúpicos apresentam acometimento de pleura e pericárdio. O acometimento peritoneal, no entanto, é especialmente raro, ocorrendo clinicamente em pequeno grupo de pacientes. O presente estudo trata do caso de uma paciente de 47 anos com diagnóstico de lúpus discoide que evoluiu com manifestações sistêmicas da doença, caracterizadas por distensão e dor abdominal significativas, astenia, emagrecimento, sinais propedêuticos de ascite e diarreia aguda baixa e não invasiva. Foi realizada exaustiva investigação diagnóstica por meio de exames laboratoriais, de imagem, colonoscopia e análise do líquido ascítico. A investigação, além de descartar a possibilidade de etiologia infecciosa, neoplásica e hemodinâmica, também permitiu a confirmação de LES. Dessa forma, a hipótese de peritonite lúpica com ascite tornou-se viável. Foi introduzido tratamento com prednisona e cloroquina, com melhora substancial do quadro.

Serositis is commonly seen in systemic lupus erythematosus (SLE). Approximately 16 percent of patients with SLE have pleural or pericardial involvement. However, peritoneal involvement is extremely rare, and clinically seen in a small group of patients. This is the case report of a 47-year old female with discoid lupus who evolved with systemic manifestations of the disease, characterized by significant abdominal distension and pain, asthenia, weight loss, signs of ascites, and acute non-invasive diarrhea. Exhaustive diagnostic investigation was performed and included laboratory and imaging tests, colonoscopy, and analysis of the ascitic fluid. Besides ruling out the possibility of an infectious, neoplastic, and hemodynamic etiology, the investigation also allowed the confirmation of SLE. Thus, the hypothesis of lupus peritonitis with ascites became viable. The patient was treated with prednisone and chloroquine, with substantial improvement of her condition.

Lupus eritematoso sistémico en varones características clínicas e inmunológicas / Systemic lupus erythematosus in men clinical and immunologic features

RESUMEN Introducción:El lupus eritematoso sistémico (LES) es una enfermedad autoinmune que afecta a mujeres nueve veces más que a los varones. La marcada influencia del género sobre la ocurrencia de LES confirma la importancia de factores genéticos y hormonales en la enfermedad. El LES en varones se caracteriza por la mayor frecuencia de serositis, severa injuria renal y un peor pronóstico. Objetivo: Determinar las características clínicas e inmunológicas de pacientes varones con LES Material y Método: Diseño observacional, descriptivo, de corte transverso. Se estudiaron pacientes con diagnóstico de LES, mayores de 18 años, internados en el Hospital Nacional de Itauguá desde enero del 2005 a diciembre del 2010. Resultados: De un total de 200 pacientes con LES, 155 fueron mujeres (77,5%) y 45 varones (22,5 %). La edad media fue 41 años. El intervalo de tiempo entre el inicio de los síntomas y el diagnóstico fue 15 meses. Características clínicas predominantes: artralgias 80%, pericarditis 60%, caída de cabello 54%, pleuritis 42%, debilidad 42%, fiebre prolongada 40 %, artritis 38%, fotosensibilidad 37%, mialgias 33%, fiebre aguda 31%. Laboratorio: anemia 93%, linfopenia 65%, leucopenia 41%, trombocitopenia 4%, ANA positivo 91%, anti-DNA positivo 68%, C3 disminuido 68%, C4 disminuido 71 %. Proteinuria mayor de 1000 mg: 35%, entre 500-1000 mg: 20%, menor a 500 mg: 45%; hematuria 46%. Se realizó biopsia renal en 22 (49%) pacientes, hallándose glomerulonefritis tipo IV (63%), tipo II (27%) y tipo III (9%). Conclusión: Las características clínicas más representativas fueron las manifestaciones articulares, seguida por las serositis (pericarditis y pleuritis) y afectación renal (grado IV). En cuanto a los marcadores inmunológicos se observó un alto porcentaje de anticuerpos antinucleares positivos además de la hipocomplementemia.

ABSTRACT Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that affects women nine times more than men. The marked influence of gender on the occurrence of SLE confirmed the importance of genetic and hormonal factors in the disease. SLE in males is characterized by a higher frequency of serositis, severe renal injury and poor outcome. Objetive: To determine the clinical and immunological features of male patients with SLE. Material an Methods: an observational, descriptive, transverse cutting, we studied 45 male patients with SLE over 18 in the National Hospital Itauguá from January 2005 to December 2010. Results: Of a total of 200 patients with SLE, 155 were women (77.5%), males 45 (22.5%), mean age was 41 years. The time interval between onset of symptoms and diagnosis was 15 months. Clinical features: 80% arthralgia, pericarditis 60%, 54% hair loss, pleuritis 42%, weakness 42%, 40% prolonged fever, arthritis 38%, photosensitivity 37%, myalgia 33%, 31% acute fever. Laboratory: anemia 93%, 65% lymphopenia, leukopenia 41%, thrombocytopenia 4%, ANA positive in 91%, anti-DNA positive 68%, decreased by 68% C3, C4 decreased by 71%. Proteinuria greater than 1000 mg. 35% between 500-1000 mg. 20%, less than 500 mg. 45%, hematuria 46%. Renal biopsy was performed in 22 (49%) patients, being glomerulonephritis type IV (63%), type II (27%) and type III (9%). Conclusion: The clinical features were more representative articular manifestations, followed by serositis (pericarditis and pleuritis) and renal (gradeIV). As for the immunological markers showed a high percentage of positive antinuclear antibodies plus hypocomplementemia.

Diagnostic value of Adenosine Deaminase (ADA) Activity inTubercular Serositis.

Background: Adenosine deaminase has been proposed to be a useful surrogate marker for tuberculosis in pleural, pericardial and peritoneal fluids. Studies have confirmed high sensitivity and specificity of Adenosine deaminase for early diagnosis of extra pulmonary tuberculosis. Aim: To assess the diagnostic level of ADA in tubercular serosal effusion and to determine its sensitivity and specificity. Methods: The study was carried out on 120 patients suffering from serosal effusion (50 pleural, 50 peritoneal, and 20 cases of pericardial effusion) . Detailed clinical history, physical examination and routine and relevant investigation of all patients including ADA estimation by GALANTI AND GIUSTI method was done. Results: ADA Level in tuberculous pleural effusion ranged from 45-160 U/L with a mean level of 100U/L and sensitivity and specificity of 100% (p<0.001, highly significant). ADA level in tuberculous peritoneal effusion ranged from 35-135 U/L with a mean level of 92U/L and sensitivity and specificity of 100% and 95% respectively (p<0.001, highly significant). ADA level in tubercular pericardial effusion ranged from 63-117 U/L with a mean level of 90U/L and sensitivity and specificity of 100% and 83.3% respectively (p<0.005, very significant).In toto serosal fluid ADA level estimation offers high degree of sensitivity and specificity of about 100% and 94.6% respectively, Conclusion: ADA was found positive with a mean value of 100U/L, 92U/L and 90 U/L in tubercular pleural, peritoneal and pericardial effusion respectively with overall 100% sensitivity and 94.6% specificity and cutoff value of 40 U/L.

A case of systemic lupus erythematosus presented with refractory serositis and nephritis during pregnancy / 대한내과학회지

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibodies and immune complex depositions, has been known that it could be aggravated during pregnancy. We report a case who developed massive pericardial effusion, pleural effusion, and proteinuria during the second trimester of pregnancy. This patient had a history of leukopenia for several years and showed several features of SLE before pregnancy, however, no specific diagnosis was made before pregnancy. High dose oral glucocorticoid with intravenous immunoglobulin administration was done, but serositis and nephritis did not improve. The induced abortion and the use of immunosuppresive agent resulted in remission. We emphasize the importance of the early diagnosis and management of SLE before pregnancy in the patients with the features of autoimmune disease such as leukopenia, skin lesion and Raynaud phenomenon for successful outcome.

A Japanese Case of Episodic Fever Compatible with Familial Mediterranean Fever / General Medicine

A 31-year-old Japanese female came to our outpatient clinic because of a 15-year history of recurrent episodic chest pain accompanied by fever, each lasting for three days. The patient was diagnosed with familial Mediterranean fever (FMF) because of the following: 1) short attacks of fever recurring at varying intervals; 2) pleuritic chest pain accompanied by fever; 3) the patient's sisters had similar episodes of fever accompanied by abdominal or chest pain; and 4) absence of any other causative factors responsible for her symptoms or pathologic findings. Although FMF has been described primarily in several limited ethnic groups, only a few cases have been reported in Japan. No diagnostic tests are commercially available for FMF so identifying the characteristic clinical picture of FMF is important.

Multiple serositis (report of five cases)

1. Five cases of multiple serositis, all caused by tuberculosis, were studied2. The absence of any marked inflammatory involvement of the abdominal organs in the cases we reported was pointed out in contrast to the almost constant marked intra-abdominal changes observed in the previously studied cases3. The relatively short duration of our cases, 2 to 10 months, was contrasted to the 2 to 4-year or even 16-year duration of the polyserositis reported by others4. A short review of the literature on multiple serositis is also given. (Summary)