Compromiso inmunológico en pacientes con síndrome de Down. Serie de casos / Immune compromise in patients with Down syndrome. A case series

El síndrome de Down, o trisomía 21, tiene una mortalidad mayor que la población general, debido principalmente a infecciones respiratorias. El objetivo de este trabajo es describir el compromiso inmunológico en una serie de casos de pacientes con síndrome de Down derivados a Inmunología por infecciones recurrentes o por hallazgo patológico de laboratorio, entre el 1 de junio de 2016 y el 31 de mayo de 2022. Se describe el compromiso de la inmunidad en 24 pacientes. Doce pacientes presentaron falla de respuesta a polisacáridos y recibieron quimioprofilaxis antibiótica y/o gammaglobulina sustitutiva. En 3 pacientes, se observó agammaglobulinemia con linfocitos B presentes y se indicó gammaglobulina sustitutiva. En 9 pacientes, se observó linfopenia T y en 1 paciente, compromiso inmune combinado.

Down syndrome, or trisomy 21, has a higher mortality than the general population, mainly due to respiratory tract infections. The objective of this study was to describe immune compromise in a series of cases of patients with Down syndrome referred to the Pediatric Immunology Section due to recurrent infections or pathological laboratory findings between 6/1/2016 and 5/31/2022. Here we describe immune compromise in 24 patients. Twelve patients failed to develop a polysaccharide response and received antibiotic chemoprophylaxis, or gamma globulin replacement therapy. Three patientsdeveloped agammaglobulinemia with presence of B cells and gamma globulin replacement therapy was indicated. Nine patients had T-cell lymphopenia and 1 patient, combined immune compromise.

Concurrencia de síndrome de Guillain-Barré y colangitis biliar primaria no vinculado a SARS-CoV-2: reporte de caso / Concurrence of Guillain-Barré syndrome and primary biliary cholangitis not related to SARS-CoV-2: case report

INTRODUCCIÓN: El síndrome de Guillain-Barré es una polirradiculoneuropatia de origen autoinmune, considerada la causa más frecuente de parálisis flácida aguda. Se han reportado diversas asociaciones del síndrome de Guillain-Barré con otras enfermedades autoinmunes no neurológicas, algunas de ellas extremadamente raras, como la que ocurre con la colangitis biliar primaria, una enfermedad crónica de etiología autoinmune cuyo diagnóstico se sustenta, además del cuadro clínico, en la alteración de las enzimas hepáticas y la presencia de anticuerpos anti-mitocondriales. CASO CLÍNICO: Paciente varón de 38 años, sin antecedente de comorbilidades previas, quien luego de presentar enfermedad diarreica dos semanas antes, desarrolló debilidad ascendente de inicio subagudo asociado a parestesias en cuatro extremidades que progresó hasta generar cuadriplejia y dificultad respiratoria. Se le realizó examen citoquímico de líquido cefalorraquídeo que evidenció disociación albumino-citológica y electromiografía que mostró hallazgos compatibles con neuropatía axonal motora aguda. Recibió tratamiento con inmunoglobulina intravenosa a dosis de 0,4 gramos por kilogramo al día, logrando mejoría del cuadro neurológico. Desde su ingreso y durante la hospitalización, presentó alteración persistente de las enzimas hepáticas que seguía un patrón colestásico. Además, se agregó dolor abdominal de leve intensidad y prurito generalizado, por lo cual fue evaluado por gastroenterología, quienes solicitaron anticuerpos anti-mitocondriales que resultaron positivos. Con esta prueba, se comprobó el diagnóstico de colangitis biliar primaria. CONCLUSIÓN: El presente caso muestra una asociación extremadamente rara de dos enfermedades autoinmunes; síndrome de Guillain-Barré y colangitis biliar primaria, tanto así que representa el primer caso reportado, no vinculado a SARS-CoV-2.

INTRODUCTION: Guillain-Barré syndrome is a polyradiculoneuropathy of autoimmune origin, considered the most frequent cause of acute flaccid paralysis. Various associations of Guillain-Barré syndrome with other non-neurological autoimmune diseases have been reported, some of them extremely rare, such as that which occurs with primary biliary cholangitis, a chronic disease of autoimmune etiology whose diagnosis is also supported by the clinical picture. , in the alteration of liver enzymes and the presence of anti-mitochondrial antibodies. CLINICAL CASE: A 38-year-old male patient, with no history of previous comorbidities, who, after presenting with diarrheal disease two weeks prior, developed subacute onset ascending weakness associated with paresthesias in four extremities that progressed to quadriplegia and respiratory distress. Cerebrospinal fluid cytochemistry was performed, which showed albuminocytological dissociation and electromyography, which showed findings compatible with acute motor axonal neuropathy, for which he received treatment with intravenous immunoglobulin at 0.4g/kg/day, achieving improvement in the neurological condition. Since admission and during hospitalization, he presented persistent changes in liver enzymes which followed a cholestatic pattern, in addition to mild abdominal pain and generalized itching, for which he was evaluated by gastroenterology, who requested anti-mitochondrial antibodies that were positive. Concluding in the diagnosis of primary biliary cholangitis. CONCLUSION: The present case shows an extremely rare association of two autoimmune diseases Guillain-Barré syndrome and primary biliary cholangitis, so much so that it represents the first case reported, not linked to SARS-CoV-2.

Relatório de recomendação para adoção de um protocolo estadual para atendimento de casos suspeitos ou confirmados de síndrome inflamatória multissistêmica em adultos (SIM-A) associada à covid-19 / Recommendation report for the adoption of a state protocol for the care of suspected or confirmed cases of multisystemic inflammatory syndrome in adults (SIM-A) associated with covid-19

Este relatório refere-se à análise crítica da Nota Técnica do Ministério da Saúde nº 38/2022-DEIDT/SVS/MS1 apresentada pela DIVE/SES/SC para a elaboração de um Protocolo Estadual de Atendimento para Casos Suspeitos ou Confirmados de Síndrome Inflamatória Multissistêmica em Adultos (SIM-A) associada à Covid-19. Na Nota Técnica emitida pelo Ministério da Saúde consta uma breve contextualização, objetivos da notificação, quadro clínico de SIM-A, definições de casos, exames complementares, exames específicos para COVID-19, manejo clínico, notificação e registro. Este relatório visa avaliar e emitir um parecer técnico embasado em evidências científicas sobre a disponibilização do medicamento Imunoglobulina Humana intravenosa (IGHIV) para o tratamento de SIM-A, fluxo de acesso ao medicamento e avaliação do impacto orçamentário, para posterior elaboração de um Protocolo Estadual para esta síndrome, destinado aos profissionais da saúde, pacientes e gestores do estado de Santa Catarina.

Miocarditis lúpica: reporte de un caso / Lupus myocarditis: a case report / Miocardite lúpica: relato de um caso

El término miocarditis hace referencia a una inflamación del miocardio, que puede tener diversas causas (infecciones, tóxicos, enfermedades autoinmunes). Su diagnóstico es desafiante debido al gran espectro de presentaciones clínicas que puede adoptar, muchas veces imitando patologías más prevalentes como el infarto agudo de miocardio. La miocarditis asociada a enfermedades autoinmunes es poco frecuente, y la importancia de reconocerla radica en que el diagnóstico e inicio temprano del tratamiento son cruciales para mejorar su pronóstico. Presentamos aquí un caso clínico de una perimiocarditis lúpica.

Myocarditis refers to an inflammation of the myocardium, which can have various causes (infections, toxic substances, autoimmune diseases). Its diagnosis is challenging due to the wide spectrum of clinical presentations, often mimicking more prevalent pathologies such as acute myocardial infarction. Myocarditis associated with autoimmune diseases is rare, and the importance of recognizing is that early diagnosis and initiation of treatment are crucial to improve its prognosis. We present here a clinical case of lupus perimyocarditis.

O termo miocardite refere-se a uma inflamação do miocárdio, que pode ter várias causas (infecções, substâncias tóxicas, doenças autoimunes). Seu diagnóstico é desafiador devido ao amplo espectro de apresentações clínicas que pode ter, muitas vezes mimetizando patologias mais prevalentes como o infarto agudo do miocárdio. A miocardite associada a doenças autoimunes é rara, e a importância de reconhecê-la reside no fato de que o diagnóstico precoce e o início do tratamento são cruciais para melhorar seu prognóstico. Apresentamos aqui um caso clínico de perimiocardite lúpica.

Errores innatos de la inmunidad en adultos / Inborn errors of immunity in adults

Introducción: La inmunodeficiencia común variable es un error innato de la inmunidad que tiene su pico de incidencia en la edad adulta. Se caracteriza por una susceptibilidad aumentada a padecer infecciones respiratorias, autoinmunidad y malignidad, secundario a un estado de hipogammaglobulinemia e inmunodisregulación, causado por mutaciones e interacciones genéticas parcialmente comprendidas. El diagnóstico es de exclusión, tiene una gran heterogeneidad clínica y comúnmente es diagnosticado de forma errónea. Objetivo: Describir un caso clínico de un paciente afectado por un error innato de la inmunidad. Caso clínico: Hombre de 35 años que se presenta a la consulta de Medicina Interna - Inmunología refiriendo un cuadro clínico de 3 años de evolución consistente en múltiples episodios de infecciones sino-pulmonares en los últimos meses, presentaba tos productiva, dificultad respiratoria y pérdida de peso no intencional de aproximadamente 8 kg. Conclusiones: La inmunodeficiencia común variable debe considerarse dentro de los diagnósticos diferenciales en todo paciente que presente alguna de sus manifestaciones clínicas, principalmente aquellas relacionadas con infecciones respiratorias a repetición, antecedente que el paciente puede presentar como relevante en sus consultas de primer nivel con medicina general o con especialistas. Su aproximación diagnóstica consiste en la solicitud de niveles séricos de inmunoglobulinas, prueba de laboratorio de fácil acceso para cualquier clínico independiente de su nivel de atención y su tratamiento se fundamenta en la administración periódica de inmunoglobulina humana exógena de forma endovenosa o subcutánea(AU)

Introduction: Common variable immunodeficiency is an inborn error of immunity that has its peak incidence in adulthood. It is characterized by an increased susceptibility to respiratory infections, autoimmunity and malignancy, secondary to a state of hypogammaglobulinemia and immunodysregulation, caused by mutations and partially understood genetic interactions. The diagnosis is one of exclusion, has great clinical heterogeneity and is commonly misinterpreted. Objective: To describe a clinical case of a patient affected by an inborn error of immunity. Methods: Retrospective description of a case report. Conclusions: Common variable immunodeficiency disorder should be considered within the differential diagnoses in every patient who presents any of its clinical manifestations, mainly those related to recurrent respiratory infections, an antecedent that the patient may present as relevant during the first-level consultations with general medicine physicians or with specialists. Its diagnostic approach consists in measuring serum immunoglobulin levels, an easily accessible laboratory test for any clinic physician regardless of their healthcare level, while its treatment is based on the periodic administration of exogenous human immunoglobulin intravenously or subcutaneously(AU)

Consenso sobre el tratamiento del síndrome inflamatorio multisistémico asociado a COVID-19 / Consensus on treatment of multisystemic inflammatory syndrome associated with COVID-19

La pandemia ocasionada por el nuevo coronavirus (SARS-CoV-2), declarada por la Organización Mundial de la Salud OMS) en marzo de 2020, afecta a un reducido número de pacientes pediátricos, quienes presentan, en su mayoría, compromiso respiratorio leve y evolución favorable. Sin embargo, en niños previamente sanos, comenzó a observarse un aumento de casos definidos como síndrome inflamatorio multisistémico (SIM-C) o similar a Kawasaki (Kawasaki-like) asociado a la enfermedad por el nuevo coronavirus (COVID-19) (KL-C) que evolucionan al shock y requieren internación en la unidad de cuidados intensivos.Los cuadros de SIM-C y los KL-C se caracterizan por fiebre, signos de inflamación, síntomas gastrointestinales y disfunción cardiovascular; las formas graves de presentación tienen mayor incidencia de hipotensión y/o shock. En el laboratorio se observan marcadores de inflamación, hipercoagulabilidad y daño miocárdico. El tratamiento farmacológico de primera línea consiste en la administración de inmunoglobulina por vía intravenosa más ácido acetilsalicílico por vía oral.Se recomienda un abordaje multidisciplinario para un diagnóstico certero y un tratamiento temprano y eficaz para disminuir la morbimortalidad.

The pandemic caused by the SARS-CoV-2 virus declared by the WHO in March 11th 2020, affects a small number of pediatric patients, who mostly present mild respiratory compromise and favorable evolution.However began to be observed in previously healthy children, an increase in cases defined as "Multisystemic Inflammatory Syndrome" (MIS-C) or "Kawasaki-like" post-COVID 19 (KL-C) that evolve to shock and require hospitalization in the Pediatric Intensive Care Unit.MIS-C and KL-C are characterized by fever; signs of inflammation, gastrointestinal symptoms, and cardiovascular dysfunction, associated with sever forms of presentation with higher incidence of hypotension and/or shock. In the laboratory, markers of inflammation, hypercoagulability and myocardial damage are observed. First-line drug treatment consists of intravenous immunoglobulin plus oral acetylsalicylic acid.A multidisciplinary approach is recommended for an accurate diagnosis and an early and effective treatment, in order to reduce morbidity and mortality.

Doença de Kawasaki: Preditores de Resistência à Imunoglobulina Intravenosa e Complicações Cardíacas / Kawasaki Disease: Predictors of Resistance to Intravenous Immunoglobulin and Cardiac Complications

Resumo Fundamento: A doença de Kawasaki (DK) é a principal causa de cardiopatia adquirida em idade pediátrica nos países desenvolvidos. Objetivos: Identificar fatores preditores de resistência à imunoglobulina intravenosa (IGIV), calcular a eficácia dos modelos preditores japoneses e caracterizar as complicações cardíacas. Métodos: Análise retrospectiva dos casos de DK entre janeiro de 2006 e julho de 2018 em um hospital pediátrico português. Foram construídas curvas ROC para encontrar fatores preditores de resistência e utilizada regressão logística multivariada para elaborar o modelo preditor. O nível de significância utilizado foi de 5%. Resultados: Foram incluídos 48 pacientes com mediana de idade de 36 meses. Verificou-se resistência à IGIV em 21%. Ocorreram alterações ecocardiográficas em 46%, com envolvimento coronário em 25%. Como variáveis preditoras de resistência, a proteína C-reativa (PC-R) apresentou uma AUC ROC = 0,789, ponto de corte = 15,1 mg/dL, sensibilidade (S) = 77,8% e especificidade (E) = 78,9%. A velocidade de sedimentação (VS) apresentou uma AUC ROC = 0,781, ponto de corte = 90,5 mm/h, S = 66,7% e E = 85,7%. O modelo com as duas variáveis apresentou valor p = 0,042 e AUC ROC = 0,790. O modelo Kobayashi apresentou S = 63,6% e E = 77,3%; Egami, S = 66,7% e E = 73,1%; e Sano, S = 28,6% e E = 94,1%. Conclusão: A PC-R e a VS são variáveis independentes que mostraram tendência preditora de resistência à IGIV com pontos de corte ótimos de 15,1 mg/dL e 90,5 mm/h, respectivamente. Cerca de metade dos pacientes teve algum tipo de envolvimento cardíaco. Os modelos japoneses não têm utilidade nessa população. (Arq Bras Cardiol. 2021; 116(3):485-491)

Abstract Background: Kawasaki disease (KD) is the leading cause of acquired cardiac disease in children, in developed countries. Objectives: To identify predictive factors for resistance to intravenous immunoglobulin (IVIG), calculate the effectiveness of Japanese predictive models and characterize cardiac complications. Methods: Retrospective analysis of KD cases admitted in a Portuguese paediatric hospital between january 2006 and july 2018. ROC curves were used to determine predictive factors for resistance and the multivariate logistic regression analysis was used to develop the predictive model. A significance level of 5% was used. Results: 48 patients with a median age of 36 months were included. The IVIG resistance was 21%. Echocardiographic anomalies were noted in 46%, with coronary involvement in 25% of the sample population. As predictive variable of resistance, the C-reactive protein (CRP) presented an AUC ROC = 0.789, optimal cut-off value 15.1 mg/dL, sensitivity (Sn) 77.8% and specificity (Sp) 78.9%. The erythrocyte sedimentation rate (ESR) presented an AUC ROC = 0.781, optimal cut-off value 90.5 mm/h, Sn 66.7% and Sp 85.7%. The model with the two variables showed p = 0.042 and AUC ROC = 0.790. Predictive strength of Japanese models were: Kobayashi (Sn 63.6%, Sp 77.3%), Egami (Sn 66.7%, Sp 73.1%), Sano (Sn 28.6%, Sp 94.1%). Conclusion: CRP and ESR are independent variables that were related to IVIG resistance, with optimal cut-off points of 15.1 mg/dL and 90.5 mm/h, respectively. About half of the patients had some form of cardiac involvement. The Japanese models appeared to be inadequate in our population. (Arq Bras Cardiol. 2021; 116(3):485-491)

Diagnostic value of the reaction at the bacillus calmette-guérin vaccination site in kawasaki disease / Utilidade diagnóstica da reação do sítio de vacinação bacillus calmette-guérin na doença de kawasaki

ABSTRACT Objective: To describe the case of an infant - diagnosed with incomplete Kawasaki disease - who developed BCG scar reactivation. Case description: A 6-month-old patient was admitted to hospital with fever associated with ocular hyperemia, cervical lymphadenopathy, and hyperemic lips, and remained hospitalized for 12 days. The physical examination revealed an inflammatory reaction at the site of the BCG scar, leading to the diagnosis of incomplete Kawasaki disease. The patient was treated with venous immunoglobulin, but presented recurrence of Kawasaki disease, with subsequent onset of coronary artery disease. Comments: BCG scar reactivation is an important finding in countries where the vaccine is routinely given and may be a useful marker for early diagnosis of Kawasaki disease, especially in its incomplete form.

RESUMO Objetivo: Descrever o caso de um lactente - com diagnóstico de Doença de Kawasaki incompleta - que desenvolveu reativação da cicatriz da vacina BCG. Descrição do caso: Um paciente de 6 meses de idade foi admitido no hospital com febre, associada à hiperemia ocular, linfadenomegalia cervical e fissuras labiais, permanecendo hospitalizado por 12 dias. Apresentava, no exame físico, reação inflamatória no local da cicatriz da vacina BCG, tendo sido feito o diagnóstico de Kawasaki incompleto. O paciente foi tratado com imunoglobulina venosa, mas apresentou recorrência da doença, com posterior surgimento de coronariopatia. Comentários: A reativação da BCG é um achado importante na doença de Kawasaki em países onde a vacina é aplicada de forma rotineira e pode ser um marcador útil para o diagnóstico precoce da doença de Kawasaki, principalmente em sua forma incompleta.

Perspectivas de tratamiento para COVID-19 (en progreso) / Treatment prospects for COVID-19 (in progress)

CONTEXTO: Debido a la contingencia por COVID-19 provocada por el nuevo coronavirus, SARS-CoV-2, en la actualidad hay una intensa investigación de alternativas terapéuticas que sean seguras y eficaces. (Hay registrados 2208 protocolos de estudios en ClinicalTrials.gov1). Con el propósito de conocer el panorama terapéutico actual contra COVID-19, se realizó una búsqueda exhaustiva de las alternativas que han demostrado cierta eficacia en esta infección, concluyendo que los estudios que se han realizado tienen limitaciones metodológicas. Se trata de estudios no controlados, con alta probabilidad de sesgos que comprometen la validez interna y externa, consideran evidencia indirecta o la experiencia de expertos ante esa emergencia sanitaria, por lo que toda recomendación derivada de estos documentos debe de tomarse con extrema cautela. El uso de esas alternativas debe considerar los riesgos y los beneficios en casos individuales, en una decisión compartida entre médicos, pacientes y familiares ya que la mayoría de la evidencia se considera de baja o muy baja calidad. A la fecha no existe tratamiento específico en contra de este virus. BÚSQUEDA REALIZADA: Inmunoglobulinas intravenosas: Las inmunoglobulinas intravenosas (IgIV) es un grupo de IgG obtenido de donantes sanos, expuestos a enfermedades infecciosas endémicas, vacunas y microorganismos ubicuos que participan en la producción de anticuerpos IgG contra diferentes microorganismos y sus productos. El uso de inmunoglobulina intravenosa se ha

Informe diário de evidências COVID-19: busca realizada em 23 de abril de 2020 / COVID-19 daily evidence report: search conducted on April 23, 2020

Essa é uma produção do Departamento de Ciência e Tecnologia (Decit) da Secretaria de Ciência, Tecnologia, Inovação e Insumos Estratégicos em Saúde (SCTIE) do Ministério da Saúde (Decit/SCTIE/MS), que tem como missão promover a ciência e tecnologia e o uso de evidências científicas para a tomada de decisão do SUS, tendo como principal atribuição o incentivo ao desenvolvimento de pesquisas em saúde no Brasil, de modo a direcionar os investimentos realizados em pesquisa pelo Governo Federal às necessidades de saúde pública. Informar sobre as principais evidências científicas descritas na literatura internacional sobre tratamento farmacológico para a COVID-19. Além de resumir cada estudo identificado, o informe apresenta também uma avaliação da qualidade metodológica e a quantidade de artigos publicados, de acordo com a sua classificação metodológica (revisões sistemáticas, ensaios clínicos randomizados, entre outros). Foram encontrados 14 artigos.

Infecção por parvovírus B19 em receptores de transplante renal: série de caso / Parvovirus B19 infection in kidney transplant recipients: a case series

Os receptores de transplante renal são mais suscetíveis a infecções, entre elas o parvovírus B19, que pode ser transmitido por via respiratória, adquirido por meio do enxerto ou por reativação de infecção latente. A anemia normocítica normocrômica, com diminuição dos reticulócitos e resistência ao tratamento com eritropoietina, é a principal forma de apresentação da infecção por parvovírus B19 em transplante renal. O diagnóstico requer alto índice de suspeição clínica e realização de testes diagnósticos selecionados. Tratamento com imunoglobulina e suspensão dos imunossupressores durante a infecção mostraram-se eficazes. Os autores relatam sua experiência com cinco casos de infecção por parvovírus B19 em receptores de transplante renal de um hospital universitário. Os aspectos clínicos, diagnósticos e terapêuticos são revistos.

Kidney transplant recipients are more susceptible to infections, including by parvovirus B19, spread through the respiratory tract, acquired through the graft or reactivation of latent infection. Normocytic normochromic anemia, with decreased reticulocytes and resistance to erythropoietin treatment, is the most common presentation of Parvovirus B19 infection in renal transplant. Diagnosis requires a higher clinical suspicion and the performance of selected diagnostic tests. Treatment with immunoglobulin and suspension of immunosuppressive therapy during the infection may be effective. The authors report five cases of PB19 infection in kidney transplant patients at a hospital. The clinical, diagnostic, and treatment features are reviewed.

Severe clinical spectrum with high mortality in pediatric patients with COVID-19 and multisystem inflammatory syndrome

OBJECTIVES: To assess the outcomes of pediatric patients with laboratory-confirmed coronavirus disease (COVID-19) with or without multisystem inflammatory syndrome in children (MIS-C). METHODS: This cross-sectional study included 471 samples collected from 371 patients (age<18 years) suspected of having severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The study group comprised 66/371 (18%) laboratory-confirmed pediatric COVID-19 patients: 61 (92.5%) patients tested positive on real-time reverse transcription-polymerase chain reaction tests for SARS-CoV-2, and 5 (7.5%) patients tested positive on serological tests. MIS-C was diagnosed according to the criteria of the Center for Disease Control. RESULTS: MIS-C was diagnosed in 6/66 (9%) patients. The frequencies of diarrhea, vomiting, and/or abdominal pain (67% vs. 22%, p=0.034); pediatric SARS (67% vs. 13%, p=0.008); hypoxemia (83% vs. 23%, p=0.006); and arterial hypotension (50% vs. 3%, p=0.004) were significantly higher in patients with MIS-C than in those without MIS-C. The frequencies of C-reactive protein levels >50 mg/L (83% vs. 25%, p=0.008) and D-dimer levels >1000 ng/mL (100% vs. 40%, p=0.007) and the median D-dimer, troponin T, and ferritin levels (p<0.05) were significantly higher in patients with MIS-C. The frequencies of pediatric intensive care unit admission (100% vs. 60%, p=0.003), mechanical ventilation (83% vs. 7%, p<0.001), vasoactive agent use (83% vs. 3%, p<0.001), shock (83% vs. 5%, p<0.001), cardiac abnormalities (100% vs. 2%, p<0.001), and death (67% vs. 3%, p<0.001) were also significantly higher in patients with MIS-C. Similarly, the frequencies of oxygen therapy (100% vs. 33%, p=0.003), intravenous immunoglobulin therapy (67% vs. 2%, p<0.001), aspirin therapy (50% vs. 0%, p<0.001), and current acute renal replacement therapy (50% vs. 2%, p=0.002) were also significantly higher in patients with MIS-C. Logistic regression analysis showed that the presence of MIS-C was significantly associated with gastrointestinal manifestations [odds ratio (OR)=10.98; 95%CI (95% confidence interval)=1.20-100.86; p=0.034] and hypoxemia [OR=16.85; 95%CI=1.34-211.80; p=0.029]. Further univariate analysis showed a positive association between MIS-C and death [OR=58.00; 95%CI=6.39-526.79; p<0.0001]. CONCLUSIONS: Pediatric patients with laboratory-confirmed COVID-19 with MIS-C had a severe clinical spectrum with a high mortality rate. Our study emphasizes the importance of investigating MIS-C in pediatric patients with COVID-19 presenting with gastrointestinal involvement and hypoxemia.

Miastenia Gravis: reporte de dos casos y revisión de la literatura / Myasthenia Gravis: two case reports and review

Myasthenia gravis is an acquired autoimmune disorder of the neuromuscular junction characterized by fluctuating weakness and fatigability of skeletal muscles. The diagnosis can be established by clinical and serologic testing, with predominance of autoantibodies against the acetylcholine receptor, and Muscle-specific kinase antibodies. We report two cases of Myasthenia gravis, the first one is a 31 year old patient with a debut of the disease, mainly with bulbar symptoms, and the second one is a 29 year old patient diagnosed with generalized Miasthenia Gravis also mainly with bulbar symptoms with worsening of symptomatology. In this report treatments alternatives and management approaches are discused

Características clínico-epidemiológicas y predictores de complicaciones coronarias en niños de Argentina con enfermedad de Kawasaki / Clinical- epidemiological characteristics and predictors of coronary complications in children of Argentina with Kawasaki disease

Resumen Introducción: La enfermedad de Kawasaki (EK) es una vasculitis sistémica aguda con riesgo de desarrollar aneurismas coronarios. Objetivos: Describir características clínico-epidemiológicas en niños con diagnóstico de EK en Argentina. Analizar factores de riesgo para el desarrollo de complicaciones coronarias (CC). Población y Métodos: Estudio multicéntrico, retrospectivo, transversal, observacional y analítico. Incluyó pacientes bajo 18 años de edad, con diagnóstico de EK en hospitales de Argentina, entre el 1 de enero de 2010 y el 31 de diciembre de 2013. Resultados: N = 193 sujetos. Edad: mediana: 29 meses. Tasa promedio total país 5 casos/10.000 egresos hospitalarios. Presentaron CC 15,5%. Mayor riesgo de CC: Mayor cantidad de días de fiebre al momento de colocación del tratamiento (p = 0,0033); Aumento de: frecuencia cardíaca (p = 0,0021), eritrosedimentación (VSG) (p = 0,005), proteína C reactiva (PCR) (p < 0,0001), leucocitosis (p = 0,0006), neutrofilia (p = 0,0021); Disminución de hematocrito (p = 0,0007) y hemoglobina (p < 0,0001). Asociación con CC: alteraciones cardiológicas no coronarias (ORv10.818); PCR mayor de 68 mg/L (OR = 11.596); leucocitos mayores a 20.000/mm3 (OR= 4.316); y VSG mayor de 64 mm/1° hora (OR = 4.267). Conclusión: La forma de presentación más frecuente fue EK completa, el riesgo de CC fue mayor en varones, menores de 5 años de edad, los factores de riesgo (clínicos y de laboratorio) fueron semejantes a los descritos en la bibliografía.

Background: Kawasaki disease (EK) is an acute systemic vasculitis with a risk of developing coronary aneurysms. Aim: To describe the clinical and epidemiological characteristics of children with EK in Argentina and to analyse the risk factors for the development of coronary's complications (CC). Methods: Multicenter, retrospective, cross-sectional, observational and analytical study. It included patients younger than 18 years of age diagnosed with EK in hospitals in Argentina, between January the 1st, 2010 and December the 31th, 2013. Results: N = 193 subjects. Age: medium: 29 months. Total incidence 5 cases / 10,000 hospital discharges. CC was observed in 15.5% of patients. Increased risk factors for CC: Elevated number of days with fever at the time of treatment placement (p = 0.0033); Increased of: heart frequency (p = 0.0021), erythrosedimentation (ESR) (p = 0.005), C-reactive protein (CRP) (p < 0.0001), leukocytes (p = 0.0006), neutrophils (p = 0.0021); Decreased of hematocrit (p = 0.0007) and hemoglobin (p < 0.0001).Association with CC: non-coronary cardiological alterations (OR = 10,818); PCR greater than 68 mg /L (OR = 11,596); leukocytes greater than 20,000 / mm3 (OR = 4.316); and ESR greater than 64 mm / 1 hour (OR = 4.267). Conclusion: The most frequent form of presentation was complete EK, the risk of CC was higher in males, younger than 5 years old, the risk factors (clinical and laboratory) were similar to those described in the literature.

Pemphigus vulgaris

Abstract: Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies

Abstract Background: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM). Main body: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Ten recommendations were developed addressing the management and therapy of systemic autoimmune myopathies. Conclusions: Robust data to guide the therapeutic process are scarce. Although not proven effective in controlled clinical trials, glucocorticoid represents first-line drugs in the treatment of SAM. Intravenous immunoglobulin is considered in induction for refractory cases of SAM or when immunosuppressive drugs are contra-indicated. Consideration should be given to the early introduction of immunosuppressive drugs. There is no specific period determined for the suspension of glucocorticoid and immunosuppressive drugs when individually evaluating patients with SAM. A key component for treatment in an early rehabilitation program is the inclusion of strengthbuilding and aerobic exercises, in addition to a rigorous evaluation of these activities for remission of disease and the education of the patient and his/her caregivers.