Dupilumabe e upadacitinibe comparados a medicamentos imunossupressores para o tratamento de dermatite atópica moderada a grave refratária a terapia tópica: revisão rápida de evidências / Dupilumab and upadacitinib compared to immunosuppressive drugs for the treatment of moderate to severe atopic dermatitis refractory to topical therapy: rapid review of evidence

Tecnologia: Dupilumabe e upadacitinibe. Comparadores: Azatioprina, metotrexato, ciclosporina, micofenolato de mofetila. Indicação: Tratamento de dermatite atópica severa em pacientes adultos. Pergunta: Dupilumabe e upadacitinibe são mais eficazes e tão seguros quanto ciclosporina ou outros agentes imunossupressores para obter os desfechos de saúde no tratamento sistêmico de dermatite atópica moderada a grave refratária à terapia atópica? Métodos: Levantamento bibliográfico foi realizado na base de dados PUBMED e Cochrane Library, seguindo estratégias de buscas predefinidas. Foi feita avaliação da qualidade metodológica das revisões sistemáticas com a ferramenta AMSTAR2 (Assessing the Methodological Quality of Systematic Reviews Version 2). Resultados: Foram selecionados três estudos que atenderam aos critérios de inclusão. Conclusão: Dupilumabe, upadacitinibe, ciclosporina e azatioprina são mais eficazes que placebo nos desfechos de eficácia (reduzir sinais clínicos em escalas, reduzir sintomas em escalas) para tratamento da dermatite atópica moderada a grave refratária à terapia tópica, mas esses medicamentos não diferem entre si. Dupilumabe, upadacitinibe, ciclosporina e azatioprina são bem tolerados e seguros

Technology: Dupilumab, upadacitinibe. Comparators: Azathioprine, methotrexate, cyclosporine, mycophenolate mofetil. Indication: Treatment of severe atopic dermatitis in adult patients. Question: Are dupilumab and upadacitinib more effective and as safe as cyclosporine or other immunosuppressive agents for achieving health outcomes in the systemic treatment of moderate to severe atopic dermatitis refractory to atopic therapy? Methods: A bibliographic survey was done in the PUBMED e Cochrane Library databases, following predefined search strategies. The methodological quality of systematic reviews was evaluated using the AMSTAR-2 tool (Assessing the Methodological Quality of Systematic Reviews Version 2). Results: Three studies that met the inclusion criteria were selected. Conclusion: Dupilumab, upadacitinib, cyclosporine, and azathioprine are more effective than placebo on efficacy endpoints (reduce clinical signs on scales, reduce symptoms on scales) for treating moderate to severe atopic dermatitis refractory to topical therapy, but these drugs do not differ from each other. Dupilumab, upadacitinib, cyclosporine, and azathioprine are well tolerated and safe

Infección por virus de epstein barr y debut de lupus eritematoso sistémico (LES) en un adolescente / Epstein barr virus infection and systemic lupus erythematosus (SLE) debut in an adolescent / Infecção pelo vírus Epstein Barr e início de lúpus eritematoso sistêmico (LES) em adolescente

Introducción: El lupus eritematoso sistémico (LES), prototipo de enfermedad autoinmune, cursa con empujes y remisiones. Dada la diversidad de presentaciones posibles, su diagnóstico y tratamiento son un reto para el clínico, y se requiere tener un alto índice de sospecha. Objetivo: presentar el caso clínico de un adolescente que debuta con LES a forma de anemia hemolítica, probablemente gatillado por infección por virus de Epstein Barr. Caso clínico: Varón de 14 años, sin antecedentes a destacar. Consulta por fiebre de 7 días de evolución de hasta 39º C, odinofagia, astenia y adinamia. Al examen físico se constata palidez cutáneo mucosa, ictericia, adenopatías cervicales y hepatoesplenomegalia. El laboratorio muestra anemia severa regenerativa con aumento de las bilirrubinas a expensas de la indirecta sin hepatitis. Prueba de Coombs positiva. Anticuerpos específicos para Epstein Barr positivos, con lo que se diagnostica anemia hemolítica secundaria a mononucleosis y se inicia tratamiento corticoideo. En la evolución agrega eritema malar y limitación en flexión de codos y rodillas. Se reciben anticuerpos antinucleares y anti ADN nativo positivos con hipocomplementemia severa. Con diagnóstico de LES se inicia hidroxicloroquina y azatioprina, manteniéndose la prednisona. Conclusiones: Muchos virus (hepatitis C, Parvovirus B19, Epstein Barr y Citomegalovirus) se han descrito como posibles inductores o simuladores de LES. Es necesario mantener un alto índice de sospecha para realizar un diagnóstico oportuno y tratamiento precoz.

Introduction: Systemic lupus erythematosus (SLE), prototype of autoimmune disease, progresses with flares and remissions. Given the diversity of possible presentations, its diagnosis and treatment are a challenge for the clinician, and a high index of suspicion is required. Objective: To present the clinical case of an adolescent who debuted with SLE in the form of hemolytic anemia, probably triggered by Epstein Barr virus infection. Clinical case: 14 - year - old male, with no history to highlight. Consultation for fever of 7 days of evolution of up to 39º C, odynophagia, asthenia and adynamia. Physical examination revealed mucous skin pallor, jaundice, cervical lymphadenopathy, and hepatosplenomegaly. The laboratory shows severe regenerative anemia with increased bilirubin at the expense of indirect without hepatitis. Positive Coombs test. Specific antibodies for Epstein Barr were positive, with which hemolytic anemia secondary to mononucleosis was diagnosed and corticosteroid treatment was started. In the evolution, it adds malar erythema and limitation in flexion of the elbows and knees. Positive antinuclear and anti-native DNA antibodies are received with severe hypocomplementemia. With a diagnosis of SLE, hydroxychloroquine and azathioprine were started, maintaining prednisone. Conclusions: Many viruses (hepatitis C, Parvovirus B19, Epstein Barr and Cytomegalovirus) have been described as possible inducers or mimics of SLE. It is necessary to maintain a high index of suspicion for timely diagnosis and early treatment.

Introdução: O lúpus eritematoso sistêmico (LES), protótipo de doença autoimune, evolui com impulsos e remissões. Dada a diversidade de apresentações possíveis, seu diagnóstico e tratamento são um desafio para o clínico, sendo necessário um alto índice de suspeição. Objetivo: apresentar o caso clínico de uma adolescente que iniciou com LES na forma de anemia hemolítica, provavelmente desencadeada por infecção pelo vírus Epstein Barr. Caso clínico: Homem de 14 anos, sem antecedentes a destacar. Consulta por febre de 7 dias de evolução de até 39º C, odinofagia, astenia e adinamia. O exame físico revelou palidez cutânea mucosa, icterícia, linfadenopatia cervical e hepatoesplenomegalia. O laboratório mostra anemia regenerativa grave com aumento da bilirrubina em detrimento da indireta sem hepatite. Teste de Coombs positivo. Anticorpos específicos para Epstein Barr foram positivos, com o qual foi diagnosticada anemia hemolítica secundária à mononucleose e iniciado tratamento com corticosteróides. Na evolução, acrescenta eritema malar e limitação na flexão dos cotovelos e joelhos. Anticorpos antinucleares e anti-DNA nativos positivos são recebidos com hipocomplementemia grave. Com diagnóstico de LES, iniciou-se hidroxicloroquina e azatioprina, mantendo-se prednisona. Conclusões: Muitos vírus (hepatite C, Parvovírus B19, Epstein Barr e Citomegalovírus) têm sido descritos como possíveis indutores ou mimetizadores do LES. É necessário manter um alto índice de suspeição para diagnóstico oportuno e tratamento precoce.

Infliximab for anoperineal lesions in Crohn's disease: remission appears to be based on rapid combination therapy at high doses

Study Aim: The aim of the present study was to compare in real life the characteristics of treatment with infliximab according to the presence or absence of anoperineal involvement in Crohn's disease. Methods: We performed a single-center, prospective, non-interventional study, on patients with Crohn's disease in remission who had been treated with infliximab for at least 1 year. Patients with poor treatment compliance, on antibiotics, or those with a stoma were excluded. Results: We included 52 patients in this study: 34 with anoperineal lesions with or without luminal lesions, and 18 with luminal lesions only. Patients with anoperineal lesions were more likely to have undergone surgery (70.6% versus 38.9%, p = 0.027), had a shorter median time to infliximab treatment initiation (0.5 versus 5.5 years, p = 0.005), a higher mean dose of infliximab (6.6 versus 5.1 mg/kg, p = 0.015), and were more likely to receive combination treatments including infliximab (52.9% versus 11.1%, p = 0.008) than patients with luminal involvement only. Conclusions In our study, infliximab treatment was initiated more quickly, at higher doses, and more in combination therapy for anoperineal Crohn's disease than for luminal damage alone. Additional studies are required to confirm this finding and to assess the tolerance of this treatment throughout patient management. (AU)

Azathioprine: its uses in dermatology

Abstract This is a narrative review of azathioprine. This medication is immunomodulatory and immunosuppressive, and it has been used widely through different medical specialties to modify disease. It has been proven useful for several dermatoses and it has encountered success when used as an off-label indication for other dermatologic diseases. Its mechanism of action is described thoroughly, as well as precautions for monitoring adequate levels in patients using it. Dermatologists should also be aware of the possible adverse events it may present. In dermatology it can be used in bullous and autoimmune diseases, and in other conditions, including intractable pruritus, atopic dermatitis, photodermatoses, psoriasis, and others. Azathioprine offers an alternative as a steroid-sparing agent and this review helps dermatologists prescribe it safely to all patients who require it.

Avaliação de pacientes com hepatite autoimune atendidos em ambulatório de referência do sul do Brasil / Evaluation of patients with autoimmune hepatitis in a specialized outpatient clinic in southern brazil

ABSTRACT BACKGROUND: Autoimmune hepatitis (AIH) is a chronic liver disease, characterized by necroinflammation and autoimmune etiology. Studies evaluating the characteristics of patients with AIH are scarce in Brazil. OBJECTIVE: Our objective was to evaluate the profile of patients with AIH in a specialized center in Southern Brazil and to verify factors related to treatment response. METHODS: this was a retrospective cohort study, which analyzed demographic, epidemiological, clinical, laboratory, and histologic data. Patients with AIH diagnosed according to the criteria of the International Autoimmune Hepatitis Group (IAIHG) were included. In liver biopsies, the degree of fibrosis, histological activity, presence of hepatocyte rosettes, plasma cell infiltrates, and confluent necrosis were evaluated. In the statistical analysis, the significance level was 5%. RESULTS: Forty adults patients diagnosed with AIH were included. The evaluated population predominantly consisted of women (75.0%) and the average age at diagnosis was 44.2 years. The association with extrahepatic autoimmune diseases occurred in 20.0% of cases. Clinically, 35.0% of patients presented with acute onset hepatitis, 37.5% with cirrhosis, and 27.5% with other forms of presentation. The most common clinical manifestation was jaundice (47.5%). Thirty-five patients were treated, and of these, 97.1% used prednisone combined with azathioprine. The average treatment time was 2.7 years. Response to treatment was complete or partial in 30 (85.7%) and absent in 5 (14.3%) patients. There was no statistically significant difference when evaluating response to treatment in relation to forms of presentation, histological findings, and the presence of autoantibodies. Regarding fibrosis, regression was observed in 18.75% of the cases. CONCLUSION: Most patients with AIH were young at presentation and of female sex. The association with extrahepatic autoimmune diseases and cirrhosis at presentation was seen in a considerable proportion of patients. Treatment was effective, but there were no clinical, histological or serological parameters capable of predicting treatment response.

RESUMO CONTEXTO: A hepatite autoimune (HAI) é uma doença hepática crônica, de caráter necroinflamatório e etiologia autoimune. Os estudos que avaliam as características de pacientes com HAI são escassos no Brasil. OBJETIVO: Nosso objetivo foi avaliar o perfil dos pacientes com HAI atendidos em um centro de referência do sul do Brasil e verificar fatores relacionados à resposta ao tratamento. MÉTODOS: Este foi um estudo de coorte retrospectivo, que analisou dados demográficos, epidemiológicos e clínicos. Nas biópsias hepáticas, foram avaliados o grau de fibrose, a atividade histológica, a presença de rosetas, de infiltrado plasmocitário e de necrose confluente. Na análise estatística, o nível de significância foi de 5%. RESULTADOS: Foram incluídos 40 pacientes adultos com diagnóstico de HAI. Houve predomínio do sexo feminino (75,0%), e a média de idade no diagnóstico foi de 44,2 anos. A associação com doenças autoimunes extra-hepáticas ocorreu em 20,0% dos casos. Clinicamente, 35,0% dos pacientes se apresentaram sob forma de hepatite aguda, 37,5% com cirrose e 27,5% com outras formas de apresentação. A manifestação clínica mais comum na apresentação foi a icterícia (47,5%). Trinta e cinco pacientes foram tratados, sendo que destes, 97,1% utilizaram prednisona associada com azatioprina. A média do tempo de tratamento foi 2,7 anos. A resposta ao tratamento foi completa ou parcial em 30 (85,7%) e ausente em 5 (14,3%) pacientes. Não houve diferença estatisticamente significativa quando avaliada a resposta ao tratamento em relação à forma de apresentação, aos achados histológicos e à presença de autoanticorpos. Em relação à fibrose, foi observada regressão em 18,75% dos casos. CONCLUSÃO: A maioria dos pacientes era jovem no momento do diagnóstico e do sexo feminino. A associação com doenças autoimunes extra-hepáticas e com cirrose na apresentação foi vista em uma parcela considerável dos casos. O tratamento foi eficaz, mas não houve parâmetros clínicos, histológicos ou sorológicos capazes de prever a resposta ao tratamento.

Hepatotoxicidade induzida por azatioprina em portadora da síndrome de Vogt-Koyanagi-Harada / Azathioprine-induced hepatotoxicity in a Vogt-Koyanagi-Harada syndrome patient

Este relato teve como objetivo apresentar um caso de hepatotoxicidade colestática induzida por azatioprina em portadora da síndrome de Vogt-Koyanagi-Harada. À admissão, apresentava icterícia +3/+4, acolia fecal e colúria, além de aumento de marcadores hepáticos, sendo compatível com síndrome colestática, cuja etiologia foi confirmada após exclusão de outras causas possíveis e retirada da azatioprina. A paciente evoluiu, após 1 semana de retirada do fármaco, com diurese livre de coloração menos escura e evacuação presente, sem acolia. Além disso, houve melhora nos exames que precederam a alta hospitalar

This report aimed at presenting a case of azathioprine-induced cholestatic hepatotoxicity in a patient with Vogt-Koyanagi-Harada syndrome. On admission, she presented with jaundice +3/+4, acholic feces, and choluria, as well as increased hepatic markers, all consistent with cholestatic syndrome, the etiology of which was confirmed after other possible causes were ruled out and azathioprine was discontinued. After 1 week of the drug discontinuation, the patient progressed with free diuresis of lighter color and defecation, with no acholia. In addition, tests performed before discharge were improved.

Informe diário de evidências COVID-19: busca realizada em 21 de maio de 2020 / COVID-19 daily evidence report: search conducted on May 21, 2020

O Informe Diário de Evidências é uma produção do Ministério da Saúde que tem como objetivo acompanhar diariamente as publicações científicas sobre tratamento farmacológico e vacinas para a COVID-19. Dessa forma, são realizadas buscas estruturadas em bases de dados biomédicas, referente ao dia anterior desse informe. Não são incluídos estudos pré-clínicos (in vitro, in vivo, in silico). A frequência dos estudos é demonstrada de acordo com a sua classificação metodológica (revisões sistemáticas, ensaios clínicos randomizados, coortes, entre outros). Para cada estudo é apresentado um resumo com avaliação da qualidade metodológica. Essa avaliação tem por finalidade identificar o grau de certeza/confiança ou o risco de viés de cada estudo. Para tal, são utilizadas ferramentas já validadas e consagradas na literatura científica, na área de saúde baseada em evidências. Cabe ressaltar que o documento tem caráter informativo e não representa uma recomendação oficial do Ministério da Saúde sobre a temática. Foram encontrados 21 artigos.

Dermatomiositis idiopática asociada a fibrosis pulmonar / Idiopathic dermatomyositis associated with pulmonary fibrosis

La incidencia de la miopatía inflamatoria idiopática es de 4 a 15 casos por cada millón de habitantes y su prevalencia de 60 por cada millón de habitantes. La dermatomiositis idiopática es más frecuente en las mujeres, aunque su asociación a fibrosis pulmonar es muy rara y solo se reporta en un 2 por ciento de los casos. Se describe el caso de un paciente de 50 años de edad, femenina, que presentó debilidad a nivel de la cintura escapular acompañada de fatiga. Tenía lesiones de rascado en diferentes regiones del cuerpo por prurito y lesiones eritematosas en la piel en ambos muslos. Además, se quejaba de dolores articulares generalizados con impotencia funcional y mialgias generalizadas progresivas e hipotrofia muscular de varios grupos musculares. El estudio analítico reveló enzimas musculares elevadas. La biopsia de piel y músculo mostró elementos sugestivos de dermatomiositis. Con la espirometría se detectó trastornos ventilatorios restrictivos de grave intensidad. Mediante la radiografía de tórax se halló infiltrado difuso peribroncovascular asociado a un trayecto fibroso y la tomografía axial computarizada precisó el pulmón con consolidación alveolar y discreto engrosamiento pleural. La paciente fue tratada con prednisona a 1 mg/kg/día asociado con azatioprina 1,5 mg/kg/día. Este tratamiento fue muy eficaz, y se logró una notable recuperación clínica y por estudios de laboratorio. Reportamos el caso de una paciente con dermatomiositis idiopática y fibrosis pulmonar. Esta asociación constituye un hallazgo infrecuente en nuestro medio y más aun con el paciente asintomático(AU)

The incidence of Idiopathic Inflammatory Myopathy is from 4 to 15 cases per million inhabitants and its prevalence of 60 per million inhabitants. Idiopathic dermatomyositis is more frequent in women; Although its association with pulmonary fibrosis is described, it is very infrequent, it is only reported in 2 percent of cases. To describe a diagnosed case of idiopathic dermatomyositis and pulmonary fibrosis. A 50-year-old patient presented weakness at the level of the shoulder girdle accompanied by fatigue. Physical examination: Skin: scratching lesions in different regions of the body due to pruritus, erythematous lesions at the level of the skin on both thighs. Osteomyoarticular system: generalized joint pains with functional impotence and progressive generalized myalgias and muscular hypotrophy of several muscle groups. The analytical study revealed elevated muscle enzymes. The skin and muscle biopsy showed elements suggestive of dermatomyositis. Chest X-ray: diffuse peribronchovascular infiltrate associated with fibrous path. Spirometry: restrictive ventilatory disorders of severe intensity. Computed tomography of the lung with alveolar consolidation and discrete pleural thickening. We report the case of a patient with idiopathic dermatomyositis and pulmonary fibrosis. This association is an uncommon finding in our environment and even more so when the patient is asymptomatic(AU)

Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes / Hepatite autoimune em 828 crianças e adolescentes brasileiros: achados clínicos e laboratoriais, perfil histológico, tratamentos e resultados

Abstract Objective: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. Methods: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments. Results: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%). Conclusions: In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.

Resumo Objetivo: Este estudo com acompanhamento de longo prazo visou a avaliar o quadro clínico, os achados laboratoriais, o perfil histológico, os tratamentos e os resultados de crianças e adolescentes com hepatite autoimune. Métodos: Foram analisados os prontuários médicos de 828 crianças e adolescentes com HAI. Foi usado um questionário para coletar os dados anônimos sobre o quadro clínico, os achados bioquímicos e histológicos e os tratamentos. Resultados: De todos os pacientes, 89,6% tinham hepatite autoimune-1 e 10,4% hepatite autoimune-2. O sexo feminino foi predominante nos dois grupos. A idade média no início dos sintomas foi 111,5 (6; 210) e 53,5 (8; 165) meses nos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente. Foi observado início clínico agudo em 56,1% e 58,8% e sintomas insidiosos em 43,9% e 41,2% dos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente. A probabilidade de insuficiência hepática foi 1,6 vezes maior para hepatite autoimune-2; 3,6% e 10,6% dos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente, apresentaram insuficiência hepática fulminante; o risco foi 3,1 vezes maior para hepatite autoimune-2. Os níveis de gamaglobulina e imunoglobulina G foram significativamente maiores nos pacientes com hepatite autoimune-1, ao passo que os níveis de imunoglobulina A e C3 foram menores em pacientes com hepatite autoimune-2; 22,4% dos pacientes apresentaram cirrose e a remissão bioquímica foi atingida em 76,2%. A taxa de sobrevida atuarial foi de 93,0%. Um total de 4,6% pacientes foram submetidos a transplante de fígado e 6,9% morreram (hepatite autoimune-1: 7,5%; hepatite autoimune-2: 2,4%). Conclusões: Nesta grande série clínica de crianças e adolescentes brasileiros, a hepatite autoimune-1 foi mais frequente e os pacientes com hepatite autoimune-2 mostraram maiores taxas de remissão da doença com respostas mais rápidas aos tratamentos. Os pacientes com hepatite autoimune-1 apresentaram maior risco de óbito.

The profile and clinical outcomes of patients with renal involvement due to IgA vasculitis: is azathioprine a good option for treatment?

Abstract Background: The Henoch-Schonlein Purpura (HSP) or IgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae. Objectives: To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. Methods: Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. Results: From the178 patients with IgA vasculitis, nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. Conclusion: Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.

Alopecia universalis associated with ulcerative colitis and the role of azathioprine

Skin manifestations can herald, co-exist, or follow the evolution of inflammatory bowel disease [IBD]. We report a middle-aged man with recent onset alopecia universalis and a history of intermittent diarrheal attacks for 6 years. Colonscopy and biopsy sampling confirmed ulcerative colitis. Regrowth of hair was achieved by treating the patient with azathioprine and mesalamine. Clinicians have to be aware that a multitude of skin manifestations with history of diarrhea can be an extraintestinal manifestation of IBD such as ulcerative colitis and this warrants further investigation

Terapia combinada en enfermedad inflamatoria intestinal: ¿una asociación necesaria? / Combination therapy in inflammatory bowel disease: a necessary association?

The management of inflammatory bowel disease (IBD) is constantly changing due to the arrival of new therapeutic agents. Combined therapy (biological associated with immunosuppressive therapy) has proven to be effective, reducing immunogenicity (antibody formation), optimizing the pharmacokinetics of biological therapy with anti-TNF. This therapeutic strategy has associated risks (neoplasia and intercurrent infections) that are not only explained by the use of drugs but also by the increase of cases in older ages. It is essential for the medical team to be familiar with the optimization and personalization of the therapy to achieve clear therapeutic objectives with the lowest possible risks.

El manejo de la enfermedad inflamatoria intestinal (EII) está en constante cambio, debido a la llegada de nuevos agentes terapéuticos. La terapia combinada (terapia biológica asociada a inmunosupresores) ha demostrado ser efectiva al disminuir la inmunogenicidad (formación de anticuerpos) permitiendo la optimización farmacocinética. Esta estrategia terapéutica tiene riesgos asociados (neoplasias e infecciones intercurrentes) que no sólo se explican por el uso de fármacos sino también por el aumento de casos en edades más avanzadas. Es fundamental que el equipo tratante este familiarizado con la optimización y personalización de la terapia para así lograr objetivos terapéuticos claros con los menores riesgos posibles.

Llega la primavera y florecen los eritemas polimorfos solares / Spring arrives and with it the polymorphous light eruption

El eritema polimorfo solar es la fotodermatosis más frecuente y suele aparecer en primavera con la primera exposición intensa al sol. Sus manifestaciones cutáneas son variadas y el diagnóstico se basa en la clínica junto al antecedente de exposición solar. En los casos leves, la fotoprotección suele ser suficiente para el control de la enfermedad, pero en formas más graves se requieren otras terapéuticas, como corticoides, antihistamínicos, o fototerapia, que genera una "fotoadaptación" de las áreas de piel afectadas. Presentamos un caso típico de erupción polimorfa solar que respondió de forma adecuada a medidas de fotoprotección. (AU)

The polymorphic solar eruption is the most frequent photodermatosis, and usually appears in spring with the first intense exposure to the sun. It has multiple cutaneous manifestations, and its diagnosis is based on the clinic and the antecedent of solar exposition. In mild cases, photoprotection is usually enough to control the disease, but in more severe forms, other therapies are required, such as corticosteroids, antihistamines, or phototherapy to generate a "photo-adaptation" of the affected skin areas. We present a typical case of polymorphic solar eruption that responded adequately to photoprotection measurements. (AU)

Orbital myosits in a patient with Behçet's disease / Miosite orbitária em paciente com doença de Behçet

Abstract The etiological diagnosis of orbital myositis (OM) is a challenge. Although it has been associated with previous infectious disease, diseases with autoimmune background, thyroidopathies or with paraneoplasic syndrome, most of them still remain as idiopathic. We describe here a case of OM uncovering a Behçet disease that is considered a rare cause for this kind of eye involvement.

Resumo Trata-se de um caso de ceratite bilateral e simultânea por Acremonium relacionada ao uso intermitente e sem respeitar a lateralidade O diagnóstico etiológico da miosite orbitária (MO) é desafiador. Embora ela tenha sido relacionada com doenças infecciosas prévias, doenças associadas à autoimunidade, tireoidopatias ou síndromes para neoplásicas, a maioria delas ainda permanece como idiopática. Descreve-se aqui, um caso de MO revelando o diagnóstico de doença de Behçet, doença considerada como uma causa pouco comum para este tipo de envolvimento ocular.

Doença de Behçet ocular: a nossa realidade / Behçet disease: our experience

Resumo Objetivo: A doença de Behçet é uma vasculite inflamatória sistémica, de etiologia desconhecida. Pode atingir virtualmente todos os sistemas, sendo as manifestações oculares comuns. O objectivo deste trabalho foi analisar doentes com doença de Behçet ocular quanto aos parâmetros demográficos, manifestações clinicas, terapêutica e principais complicações. Métodos: Estudo descritivo e retrospetivo, que incluiu 11 doentes com o diagnóstico de doença de Behçet ocular, segundo os critérios do ISG, observados na consulta de Inflamação Ocular do Hospital Prof. Doutor Fernando da Fonseca nos últimos 3 anos. Resultados: Identificaram-se 11 doentes, 5 homens e 6 mulheres, todos caucasianos. A idade média ao diagnóstico foi de 33,45±6,49 anos. A manifestação ocular foi o primeiro sinal da doença em 2 doentes. Em 72,7% dos casos as manifestações oculares foram bilaterais. Identificaram-se 4 casos de panuveíte, 3 de uveíte posterior, 2 de uveíte anterior, 1 de queratite e 1 caso de episclerite. O glaucoma e a catarata foram a complicação ocular mais frequente. O tratamento sistémico incluiu a corticoterapia oral em associação com terapêutica adjuvante imunossupressora, sendo os mais utilizados a azatioprina e a ciclosporina. Em 3 doentes houve necessidade de terapêutica biológica com infliximab para controlo da doença. Conclusão: A manifestação ocular mais frequente foi a panuveíte. Esta doença pode condicionar complicações oculares com diminuição irreversível da acuidade visual. A orientação destes doentes exige uma abordagem global e interdisciplinar.

Abstract Objective: Behçet's disease is a systemic inflammatory vasculitis of unknown etiology. It can virtually reach all systems, being common ocular manifestations. The aim of this study was to analyze patients with ocular Behcet's disease regarding demographic parameters, clinical manifestations, therapeutic approach and main complications. Methods: Descriptive and retrospective study, including 11 patients diagnosed with ocular Behcet's disease, according to the ISG criteria, observed in the Ocular Inflammation appointment of the Hospital Prof. Doutor Fernando da Fonseca in the last 3 years. Results: We identified 11 patients, 5 males and 6 females, all caucasian. The mean age at diagnosis was 33.45 ± 6.49 years. Ocular manifestation was the first sign of the disease in 2 patients. In 72.7% of the cases, ocular manifestations were bilateral. There were four cases of panuveitis, 3 of posterior uveitis, 2 of anterior uveitis, 1 of keratitis and 1 case of episcleritis. Glaucoma and cataract were the most frequent ocular complications. Systemic treatment included oral corticosteroid therapy in combination with adjuvant immunosuppressive therapy, and the most commonly used was azathioprine and cyclosporine. In 3 patients there was a need for biological treatment with infliximab to control the disease. Conclusion: The most frequent ocular manifestation was panuveitis. This disease can promote eye complications with an irreversible decrease in visual acuity. Orientation of these patients requires a global and interdisciplinary approach.

Hemangioendotelioma epitelioide pulmonar: Reporte de un caso / Lung epithelioid hemangioendothelioma: Report of one case

Epithelioid hemangioendothelioma is a multifocal tumor that rarely metastasizes. It is difficult to diagnose, most often it is an incidental finding in young asymptomatic women. The radiologic pattern is heterogeneous. Histologic confirmation of Weibel-Palade bodies or immunohistochemistry based on specific tumor markers such as factor VIII and CD34 are the most important finding to confirm the diagnosis. We report a 21 years old woman Presenting with cough and dyspnea. A chest X ray was suggestive of tuberculosis. Sputum smears were negative for acid fat bacilli and the tuberculin test was negative. A chest CAT scan showed multiple nodular lesions. A surgical biopsy of the lesions confirmed the presence of a hemangioendothelioma. The patient was initially treated with prednisone and azathioprine without response. Thereafter, the patient is without treatment and without evidence of disease progression.

Coroidopatia no lúpus eritematoso sistêmico / Choroidopathy in systemic lupus erythematosus

Resumo O Lúpus Eritematoso sistêmico (LES) é uma doença autoimune que pode afetar o olho de diversas formas. A coroidopatia lúpica é rara e apresenta-se com descolamento seroso de retina, descolamento do epitélio pigmentar da retina (EPR) e epiteliopatia pigmentar. A maioria dos casos está associada à atividade sistêmica da doença, podendo ser considerada um fator de gravidade e necessidade de imunossupressão intensa. Geralmente apresenta bom prognóstico visual com o tratamento adequado do LES, apesar de alguns casos evoluírem com danos irreversíveis na retina externa e EPR. Descrevemos um caso de coroidopatia secundaria ao LES com atividade multisistêmica com boa evolução após tratamento clínico com imunossupressão sistêmica.

Abstract Systemic lupus erythematosus (SLE) is an autoimmune disease in which can affect the eye in different ways. Lupus choroidopathy is rare and include retinal pigment epithelium (RPE) detachment and/or serous retinal detachment and pigment epitheliopathy. Most cases are associated with systemic disease activity and can be considered a factor of gravity and need for intense immunosuppression. Usually has good visual prognosis with proper treatment of SLE, although some cases may have irreversible damage to the outer retina and RPE. We describe a case of choroidopathy secondary to SLE during its multisystem activity with good clinical outcome after treatment with systemic immunosuppression.