Síndrome de Vogt-Koyanagi-Harada

El síndrome de Vogt-Koyanagi-Harada es una enfermedad autoinmune multisistémica crónica, caracterizada por panuveítis difusa granulomatosa bilateral con desprendimiento exudativo de retina y papilitis. Compromete el sistema nervioso central (meninges, disacusia neurosensorial) así como piel y mucosas. A pesar de ser una enfermedad compleja y poco frecuente, se hace necesario comprender la importancia del diagnóstico rápido y el tratamiento oportuno con seguimiento especializado. Es por ello que se decidió realizar una revisión de la literatura con el objetivo de actualizar los conocimientos existentes sobre este tema. La búsqueda se realizó en diferentes publicaciones y textos básicos de la especialidad. Las fuentes consultadas fueron las bases de datos PubMed y Google Scholar. El diagnóstico de la enfermedad es esencialmente clínico y son los oftalmólogos quienes más lo sospechan por ser los síntomas oculares los más frecuentes y dramáticos. El pronóstico visual de los pacientes es generalmente bueno si el diagnóstico es precoz y se indica un tratamiento adecuado. Los corticosteroides sistémicos a altas dosis asociados a inmunosupresores y agentes biológicos tienen gran impacto en la evolución de la enfermedad, sobre todo estos últimos a nivel mundial, previniendo complicaciones y permitiendo resultados visuales satisfactorios para una mejor calidad de vida del paciente.

Vogt-Koyanagi-Harada syndrome is a chronic multisystem autoimmune disease characterized by bilateral diffuse granulomatous panuveitis with exudative retinal detachment and papillitis. It involves the central nervous system (meninges, sensorineural dysacusis) as well as skin and mucous membranes. In spite of being a complex and infrequent disease, it is necessary to understand the importance of rapid diagnosis and timely treatment with specialized follow-up. For this reason, it was decided to carry out a review of the literature with the aim of updating the existing knowledge on this subject. The search was carried out in different publications and basic texts of the specialty. The sources consulted were the PubMed and Google Scholar databases. The diagnosis of the disease is essentially clinical and it is the ophthalmologists who suspect it the most because the ocular symptoms are the most frequent and dramatic. The visual prognosis of patients is generally good if the diagnosis is early and adequate treatment is indicated. Systemic corticosteroids at high doses associated with immunosuppressants and biological agents have a great impact on the evolution of the disease, especially the latter worldwide, preventing complications and allowing satisfactory visual results for a better quality of life of the patient.

Clinical profile and management with immunosuppressants and biologics in Behcet’s uveitis: A cohort of 25 patients from a tertiary eye care center in South India

Purpose: To report the clinical profile of Behcet’s disease and its management with immunosuppressants and biologics in a cohort of 25 patients from a tertiary eye care center in South India. Methods: This was a retrospective, observational study. Records of 45 eyes of 25 patients between January 2016 and December 2021 were retrieved from the hospital database. Complete ophthalmic evaluation and systemic examination by the rheumatologist with appropriate investigations had been done. Results were analyzed using Statistical Package for the Social Sciences (SPSS) software. Results: Males (19, 76%) were found to be more affected than females (6, 24%). Mean age of presentation was 27.68 ± 11.08 years. Twenty patients had bilateral involvement (80%), and unilateral involvement was seen in five patients (20%). Seven eyes of four patients (16%) had isolated anterior uveitis, out of which one patient had unilateral and three patients had bilateral involvement. Twenty?six eyes of 16 patients (64%) had posterior uveitis, out of which six patients had unilateral and 10 had bilateral involvement. Twelve eyes of seven patients (28%) had panuveitis, out of which two patients had unilateral and five had bilateral involvement. Hypopyon was seen in five eyes (11.1%) and posterior synechiae in seven eyes (15.55%). Posterior segment findings included vitritis (24.44%), vasculitis (17.78%), retinitis (17.78%), disc hyperemia (11.11%), and disc pallor (8.89%). Steroids alone were given in five patients (20%) and intravenous methylprednisolone (IVMP) was given in four patients (16%). Immunosuppressive agents along with steroids were given in 20 patients (80%), of which azathioprine alone was given in seven patients (28%), cyclosporin alone was given in two patients (8%), mycophenolate mofetil alone was given in three patients (12%), combination of azathioprine and cyclosporin was given in six patients (24%), and combination of methotrexate and mycophenolate mofetil was given in one patient (4%). Biologics were given in 10 patients (40%) – adalimumab in seven patients (28%) and infliximab in three patients (12%). Conclusion: Behcet’s disease is an uncommon uveitis in India. Addition of immunosuppressants and biologics to conventional steroid therapy gives better visual outcomes.

Application of machine learning in the therapeutic drug monitoring and individual drug therapy / 中国药房

Machine learning has been applied in the medical field due to its powerful data analysis and exploration capabilities. In recent years， more and more studies have applied it to therapeutic drug monitoring and individual drug therapy of immunosuppressants， anti-infective drugs， antiepileptic drugs， etc. Compared with the traditional population pharmacokinetic modeling methods， the constructed models based on machine learning can predict blood drug concentration and drug dose more accurately， improve the level of clinical precision drug use and reduce the occurrence of adverse drug reactions. Based on this， this article reviews the application of machine learning in therapeutic drug monitoring and individual drug therapy， with a view to providing theoretical basis and technical support for clinical precise drug use.

Prevalência de síndrome metabólica em pacientes pós-transplante renal / Prevalence of metabolic syndrome in post-kidney transplantation patients

O desenvolvimento da síndrome metabólica após o transplante renal (TxR) é evidenciado na literatura e se caracteriza por alterações que conferem a redução da função do enxerto. Objetivo: O objetivo deste trabalho é descrever a prevalência da síndrome metabólica e identificar variáveis que se associam à síndrome metabólica em receptores de TxR tardio. Metodologia: Trata-se de um estudo transversal analítico realizado com pacientes receptores de TxR. Foram incluídos participantes com idade superior ou igual a 18 anos, receptores de TxR tardio em acompanhamento superior a 6 meses. A análise descritiva dos dados foi expressa por médias, desvio padrão, medianas e percentuais. A normalidade dos dados foi verificada pelo teste de Shapiro-Wilk ao nível de 5% de significância. Aplicou-se o teste de comparação de médias para duas amostras independentes, teste t de Student. Foi utilizado o teste de correlação de Pearson para avaliar as possíveis relações existentes entre a variável tempo pós TxR com as outras variáveis de interesse. Resultados: Ao avaliar 43 pacientes com idade média de 50,9 ± 9,8 anos, notou-se que a síndrome metabólica esteve presente em 53,85% dos voluntários e que se associou ao peso prévio (p= 0,018), concentrações séricas de triglicerídeos (p= 0,001), menores médias de HDL (p= 0,053) e, também, foi verificada uma associação marginal com a circunferência da cintura (p= 0,051). A SM foi prevalente no período pós TxR. Ainda, as maiores médias de peso prévio ao TxR, de circunferência da cintura, de triglicerídeos e as menores médias de HDL se associaram à SM

The onset of metabolic syndrome (MS) after kidney transplantation (KTx) is evidenced in the literature and this is characterized by alterations that confer a reduction in the function of the transplantation. Objective: The aim of this research is to describe the prevalence of the metabolic syndrome and to identify the variables that are associated with the metabolic syndrome in recipients of belated KTx. Methods: This is an analytical cross-sectional study carried out with patients receiving KTx. Participants aged 18 years or older, recipients of late KTx with follow-up longer than 6 months, were included. The descriptive analysis of the data was expressed as means, standard deviation, medians and percentages. Data normality was verified using the Shapiro-Wilk test at a 5% significance level. The mean comparison test was applied for two independent samples, Student's t test. Pearson's correlation test was used to assess possible association between the variable time after KTx and the other variables of interest. Results: Forty-three patients with a mean age of 50.9 ± 9.8 years were evaluated and metabolic syndrome was present in 53.85% of the volunteers. There was an association with previous weight (p= 0.018), serum triglyceride concentrations (p= 0.001) and lower HDL means (p= 0.053) and and a marginal association with waist circumference (p=0,0051). MS was prevalent in the post-KTx period. Furthermore, the highest means of weight prior to KTx, waist circumference, TG and the lowest means of HDL were associated with MS

Actividad del sistema inmune en pacientes reumáticos

Las enfermedades reumáticas son un grupo de afecciones, la mayoría de ellas con afectación inmunológica y compromiso sistémico, que generan distintos grados de discapacidad funcional y disminución de la percepción de calidad de vida de los pacientes que las padecen. Constituyen una de las primeras causas de discapacidad, de invalidez temporal y definitiva, de afectación laboral y se sitúan dentro de los 10 primeros motivos de consultas en el primer nivel de atención de salud. Los pacientes con este tipo de enfermedad han sido considerados como pacientes inmunodeprimidos lo cual no se corresponde directamente con los mecanismos de producción de la gran mayoría de las enfermedades reumáticas. En el presente reporte se abordan los mitos y realidades que engloban a las enfermedades reumáticas y la supuesta inmunosupresión que generan, desde la perspectiva etiopatogénica. El esclarecimiento de estos elementos es indispensable para poder enfrentar adecuadamente, desde el punto de vista terapéutico y de autocuidado a los pacientes con enfermedades reumáticas. Se concluye, que con base en los elementos etiopatogénicos de las enfermedades reumáticas y el análisis de la expresión de la respuesta inmunológica normal y en pacientes reumáticos, estos no pueden ser considerados como personas inmunodeprimidas. La prescripción de inmunosupresores se realiza con la finalidad de restaurar la respuesta inmunológica normal.

Rheumatic diseases are a group of conditions, most of them with immunological affectation and systemic compromise, which generate different degrees of functional disability and decrease in the perception of quality of life of the patients who suffer from them. They constitute one of the main causes of disability, of temporary and definitive invalidity, of labor affectation and are among the first 10 reasons for consultations in the first level of health care. Patients with this type of disease have been considered immunocompromised, which does not correspond directly to the mechanisms of production of the vast majority of rheumatic diseases. This report addresses the myths and realities that encompass rheumatic diseases and the supposed immunosuppression they generate, from the etiopathogenic perspective of this group of diseases. The clarification of these elements is essential to be able to deal adequately, from the therapeutic and self-care point of view, with patients with rheumatic diseases. It is concluded that, based on the etiopathogenic elements of rheumatic diseases and the analysis of the expression of the normal immune response and in rheumatic patients, these cannot be considered as immunosuppressed people. The prescription of immunosuppressants is made in order to restore the normal immune response.

Construção e validação de cartilha educativa sobre o uso de imunossupressores no pós transplante renal / Construction and validation of an educational booklet on the use of immunosuppressive drugs after kidney transplantation / Construcción y validación de folleto educativo sobre el uso de inmunosupresores en el postrasplante renal

RESUMO Objetivo: construir e validar por concordância uma cartilha educativa sobre o uso dos imunossupressores no pós-transplante renal. Método: estudo metodológico, realizado em um ambulatório de acompanhamento de pacientes pós transplantados, no período de dezembro de 2019 a agosto de 2020. Desenvolvido em duas fases: construção da cartilha e validação por concordância do material por juízes especialistas. Foi considerado o nível de concordância de, no mínimo, 80% para se garantir a validação do material. Resultados: dos 30 itens avaliados da cartilha, na primeira fase, 15 foram modificados. Após as modificações do material, na segunda fase, somente um item apresentou valor de concordância inferior a 80%. Conclusão: a cartilha foi validada do ponto de vista de conteúdo e aparência, possibilitando auxílio aos profissionais de saúde no processo de educação em saúde, sendo um alicerce na assistência prestada aos pacientes transplantados renais.

ABSTRACT Objective: Construct and validate by agreement an educational booklet on the use of immunosuppressive drugs after kidney transplantation. Method: Methodological study carried out in an outpatient follow-up clinic for post-transplant patients, from December 2019 to August 2020. It was developed in two phases: construction of the booklet and validation by agreement of the material by expert judges. A minimum level of agreement of 80% was necessary for the validation of the material. Results: Of the 30 items evaluated in the booklet, in the first phase, 15 were modified. After the modifications in the material performed in the second phase, only one item had an agreement value lower than 80%. Conclusion: The booklet was validated in content and appearance. Thus, the material could be used to assist health professionals in the health education process and support the care provided to kidney transplant patients.

RESUMEN Objetivo: construir y validar por concordancia un folleto educativo sobre el uso de los inmunosupresores en el postrasplante renal. Método: estudio metodológico, realizado en un ambulatorio de seguimiento de pacientes post-trasplantados, en el periodo diciembre de 2019 a agosto de 2020. Desarrollado en dos fases: construcción del folleto y validación por concordancia del material por jueces especialistas. Se consideró un nivel de concordancia de al menos un 80% para garantizarse la validación del material. Resultados: de los 30 ítems evaluados del folleto en la primera fase, 15 fueron modificados. Después de las modificaciones del material, en la segunda fase, sólo un ítem presentó valor de concordancia inferior a 80%. Conclusión: el folleto fue validado desde el punto de vista del contenido y apariencia, posibilitando auxilio a los profesionales de la salud en el proceso de educación en salud, siendo una piedra angular en la asistencia prestada a los pacientes trasplantados renales.

Manifestaciones digestivas en el curso de enfermedades reumáticas, estudio de 5 años / Digestive manifestations in the course of rheumatic diseases, 5-year study

RESUMEN Introducción: Las enfermedades reumáticas son un grupo de alrededor de 250 afecciones que afectan fundamentalmente el sistema osteomioarticular, aunque la mayoría tiene un carácter sistémico. En el caso del sistema digestivo el compromiso puede estar causado por el propio proceso inflamatorio de la enfermedad o secundario a la afectación que causa los distintos grupos farmacológicos usados contra las enfermedades reumáticas. Objetivo: Caracterizar el compromiso digestivo de una cohorte de paciente con enfermedades reumáticas durante un periodo de estudio de 5 años. Métodos: Investigación básica, no experimental, descriptiva y longitudinal que incluyó como población de estudio una cohorte de 109 pacientes con diagnóstico de enfermedades reumáticas según criterios del Colegio Americano de Reumatología. Se identificaron las características generales de los pacientes, de las enfermedades reumáticas incluidas en la investigación y las principales manifestaciones digestivas identificadas. Resultado: Promedio de edad de 57,83 años, predominio de pacientes femeninas (71,56 %), procedencia urbana (55,05 %) y con diagnóstico de artritis reumatoide (50,46 %). El 90,82 % de los pacientes refirió haber utilizado glucocorticoides. El 94,49 % de los pacientes presentó algún tipo de manifestación extraarticular digestiva. La gastritis (36,89 %), úlcera péptica (25,24 %), reflujo gastroesofágico (18,45 %) y sequedad bucal (15,53 %) fueron las de mayor frecuencia de presentación. Conclusiones: Las manifestaciones digestivas constituyen una expresión extraarticular frecuente en el curso de las enfermedades reumáticas. Su origen pude estar supeditado a la patogenia de la enfermedad o a los eventos adversos de los grupos farmacológicos utilizados en los esquemas terapéuticos de las enfermedades reumáticas. Los glucocorticoides fueron el grupo farmacológico más usado. Algunas manifestaciones digestivas puede llegar a poner en peligro la vida de los pacientes.

ABSTRACT Introduction: Rheumatic diseases are a group of around 250 conditions that mainly affect the osteomyoarticular system, but most of them have a systemic nature, which is why they affect different organs and organ systems. In the case of the digestive system, the compromise may be caused by the inflammatory process of the disease itself or secondary to the affectation generated by the different pharmacological groups that are used to control rheumatic diseases. Objective: To characterize the digestive compromise of a cohort of patients with rheumatic diseases during a study period of 5 years. Methods: Basic, non-experimental, descriptive, correlational and longitudinal research that included as a study population a cohort of 109 patients diagnosed with rheumatic diseases according to the criteria of the American College of Rheumatology. The general characteristics of the patients, the rheumatic diseases included in the research, the main digestive manifestations identified were identified. Result: Average age of 57.83 years, predominance of female patients (71.56%), urban origin (55.05%) and with a diagnosis of rheumatoid arthritis (50.46%). 90.82% of the patients reported having used glucocorticoids. 94.49% of the patients presented some type of extra-articular digestive manifestation. Gastritis (36.89%), peptic ulcer (25.24%), gastroesophageal reflux (18.45%) and dry mouth (15.53%) were the ones with the highest frequency of presentation. Conclusions: Digestive manifestations are a frequent extra-articular expression in the course of rheumatic diseases. Its origin may be subject to the etiopathogenic mechanisms of the disease or to the adverse events of the pharmacological groups used in the therapeutic regimens for rheumatic diseases. Glucocorticoids were the most frequently used pharmacological group in the therapeutic regimens for rheumatic diseases. The presence of some digestive manifestations endanger the lives of patients.

Construção e validação de cartilha educativa sobre o uso de imunossupressores no pós transplante renal / Construction and validation of an educational booklet on the use of immunosuppressive drugs after kidney transplantation / Construcción y validación de folleto educativo sobre el uso de inmunosupresores en el postrasplante renal

RESUMO Objetivo: construir e validar por concordância uma cartilha educativa sobre o uso dos imunossupressores no pós-transplante renal. Método: estudo metodológico, realizado em um ambulatório de acompanhamento de pacientes pós transplantados, no período de dezembro de 2019 a agosto de 2020. Desenvolvido em duas fases: construção da cartilha e validação por concordância do material por juízes especialistas. Foi considerado o nível de concordância de, no mínimo, 80% para se garantir a validação do material. Resultados: dos 30 itens avaliados da cartilha, na primeira fase, 15 foram modificados. Após as modificações do material, na segunda fase, somente um item apresentou valor de concordância inferior a 80%. Conclusão: a cartilha foi validada do ponto de vista de conteúdo e aparência, possibilitando auxílio aos profissionais de saúde no processo de educação em saúde, sendo um alicerce na assistência prestada aos pacientes transplantados renais.

ABSTRACT Objective: Construct and validate by agreement an educational booklet on the use of immunosuppressive drugs after kidney transplantation. Method: Methodological study carried out in an outpatient follow-up clinic for post-transplant patients, from December 2019 to August 2020. It was developed in two phases: construction of the booklet and validation by agreement of the material by expert judges. A minimum level of agreement of 80% was necessary for the validation of the material. Results: Of the 30 items evaluated in the booklet, in the first phase, 15 were modified. After the modifications in the material performed in the second phase, only one item had an agreement value lower than 80%. Conclusion: The booklet was validated in content and appearance. Thus, the material could be used to assist health professionals in the health education process and support the care provided to kidney transplant patients.

RESUMEN Objetivo: construir y validar por concordancia un folleto educativo sobre el uso de los inmunosupresores en el postrasplante renal. Método: estudio metodológico, realizado en un ambulatorio de seguimiento de pacientes post-trasplantados, en el periodo diciembre de 2019 a agosto de 2020. Desarrollado en dos fases: construcción del folleto y validación por concordancia del material por jueces especialistas. Se consideró un nivel de concordancia de al menos un 80% para garantizarse la validación del material. Resultados: de los 30 ítems evaluados del folleto en la primera fase, 15 fueron modificados. Después de las modificaciones del material, en la segunda fase, sólo un ítem presentó valor de concordancia inferior a 80%. Conclusión: el folleto fue validado desde el punto de vista del contenido y apariencia, posibilitando auxilio a los profesionales de la salud en el proceso de educación en salud, siendo una piedra angular en la asistencia prestada a los pacientes trasplantados renales.

Progress on pathogenesis and treatment of ocular cicatricial pemphigoid / 国际眼科杂志(Guoji Yanke Zazhi)

@#Ocular cicatricial pemphigoid(OCP)is a special manifestation of mucosal pemphigoid(mucous membrane pemphigoid, MMP), and the pathogenesis is not clear at present. It can be caused by variety factors such as antigen-antibody reaction, inflammation cell infiltration, the action of various cytokines, elevated calcium ion levels and susceptibility genes. In the early period of diseases, conjunctival present chronic progressive fibrotic inflammation, later the corneal opacity and the neovascularization will eventually lose vision. Therefore, it is particularly important to carry out clinical standardized treatment for OCP patients in a timely manner. To use medicine to control the inflammation and delay the progression of the disease, for example, dapsone, intravenous immunoglobulin(IVIG), rituximab(RTX), tumor necrosis factor antagonists and adrenocorticoids. Surgical treatment can be considered appropriately when OCP patients are complicated by severe trichiasis, corneal disease and cataract.

Research Advance in Treatment of Thrombotic Thrombocytopenic Purpura --Review / 中国实验血液学杂志

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy, in which a severe deficiency of von Willebrand factor lyase results in thrombocytopenic clots that block blood vessels and eventually lead to terminal organ failure. Therapeutic plasma exchange is the cornerstone of TTP treatment which can greatly improves the survival rate of the patients. With the further exploration to the pathophysiological mechanism of TTP, other alternative therapies, new immunosuppressive agents, targeted antagonists, gene therapy and other emerging means gradually emerge, which are expected to further reduce the mortality and recurrence rate of the patients. In this review, the new developments in TTP treatment were summarized briefly.

Mastitis granulomatosa idiopática: Caso clínico y revisión literaria / Idiopathic granulomatose mastitis: Case report and literary review

Se describe el caso clínico de una paciente con diagnóstico histológico de mastitis granulomatosa idiopática, enfermedad poco frecuente en la que se utilizan inmunosupresores como tratamiento descripto y puede confundirse con otras entidades atendidas en la especialidad. Se describe el caso y se realiza una revisión de la literatura.

The clinical case of a patient with a histological diagnosis of idiopathic granulomatous mastitis is described, a rare disease in which immunosuppressants are used as the described treatment and can be confused with other entities treated in the specialty. The case is described and a literature review is carried out.

Surviving complications of retroperitoneal fibrosis with intermittent relapses over a period of 20 years: case report and literature review / Sobrevivir a las complicaciones de la fibrosis retroperitoneal con recaídas intermitentes durante un período de 20 años: reporte de un caso y revisión de la literatura

Idiopathic retroperitoneal fibrosis is a rare fibro-inflammatory disease of varied etiology which usually originates around aorta and spreads caudally along Iliac vessels into adjacent retroperitoneum causing ureteral obstruction as the most frequent complication.A 53-year-old male patient presented with complaint of mild pain in both the legs off and on. On investigating further, we found that he had been struggling with intermittent relapses every 3-4 years for last 20 years since he was first diagnosed with Idiopathic Retroperitoneal Fibrosis. He was 33-year-old when he first developed the symptoms of anuria for 48 hours and was diagnosed with Idiopathic retroperitoneal fibrosis. This was followed by atrophy of left kidney and hypertension 6 years later, then hypothyroidism after another 3years and finally involvement of Inferior Vena Cava and acute Deep Vein Thrombosis of lower limbs after another 3-4 years. His deep vein thrombosis was well managed in time. He was put on glucocorticoids everytime he had a relapse and a complication.We did a review of literature to understand recent advances about its pathogenesis, diagnosis, investigations and management. We searched in PubMed using terms like retroperitoneal fibrosis alone and in combination with related terms such as Inferior Vena Cava thrombosis, Deep Vein Thrombosis, Tamoxifen, Methotrexate. This case is unique as it is very rare to find acute Deep Vein Thrombosis in Idiopathic retroperitoneal fibrosis without development of any collaterals when Inferior Vena Cava lumen is compromised to almost complete obstruction.After a follow up of 20 years patient is doing well in terms of physical activity and psychological wellbeing with anti-hypertensives, thyroxine and anti-coagulants. Is the disease-free interval actually free of the disease or it just subsided with immunosuppressants to become active after some time?

La fibrosis retroperitoneal idiopática es una enfermedad fibroinflamatoria rara, de etiología variada que generalmente se origina alrededor de la aorta y se propaga caudalmente a lo largo de los vasos ilíacos en retroperitoneo adyacente causando obstrucción ureteral como la complicación más frecuente.Reportamos el caso de un paciente varón de 53 años que se presentó con un dolor leve en ambas piernas. Al investigar más a fondo, descubrimos que había estado luchando con recaídas intermitentes cada 3-4 años durante los últimos 20 años desde que se le diagnosticó por primera vez fibrosis retroperitoneal idiopática. Tenía 33 años cuando desarrolló por primera vez los síntomas de anuria durante 48 horas y se le diagnosticó fibrosis retroperitoneal idiopática. Esto fue seguido por atrofia del riñón izquierdo e hipertensión 6 años después, luego hipotiroidismo después de otros 3 años y finalmente afectación de la vena cava inferior y trombosis venosa profunda aguda de las extremidades inferiores después de otros 3-4 años. Su trombosis venosa profunda se controló bien a tiempo. Le recetaron glucocorticoides cada vez que tenía una recaída y una complicación.Hicimos una revisión de la literatura para comprender los avances recientes sobre su patogenia, diagnóstico, investigaciones y manejo. Se realizaron búsquedas en PubMed utilizando términos como fibrosis retroperitoneal sola y en combinación con términos relacionados como trombosis de la vena cava inferior, trombosis venosa profunda, tamoxifeno, metotrexato. Este caso es único, ya que es muy raro encontrar trombosis venosa profunda aguda en fibrosis retroperitoneal idiopática sin desarrollo de colaterales cuando la luz de la vena cava inferior está comprometida hasta una obstrucción casi completa.Después de un seguimiento de 20 años, el paciente se encuentra bien en términos de actividad física y bienestar psicológico con antihipertensivos, tiroxina y anticoagulantes. ¿El intervalo libre de enfermedad está realmente libre de la enfermedad o simplemente disminuyó con inmunosupresores para activarse después de algún tiempo?

Neutrophil-to-lymphocyte ratio in systemic lupus erythematosus is influenced by steroids and may not completely reflect the disease activity

Objectives: Neutrophil-to-lymphocyte ratio (NLR) has emerged as an important parameter in inflammatory network andcould pave the way for newer treatment strategies in systemic lupus erythematosus (SLE). The study evaluated NLR as apredictor of disease activity in SLE and attempted to relate the factors influencing the NLR.Methods: The study included 117 SLE patients fulfilling the systemic lupus international collaborating clinics/AmericanCollege of Rheumatology (SLICC/ACR) criteria (2010). The subjects were classified into mild, moderate, and severesystemic lupus erythematosus disease activity index 2000 (SLEDAI 2K) groups and compared. NLR values were classifiedas ≤2, >2–4 and >4 groups and its relationship with study variables was evaluated by Notched box-and-Whisker plots,Spearman correlation and Mountain plot. ROC and multiple linear regression were used to verify discriminatory abilityand factors influencing NLR respectively.Results: Approximately 24% (n=28) of patients each had mild and moderate SLEDAI disease activities, and 52.14% (n=61)had severe activity. Patients with severe disease activity were significantly younger (31.69±10.09 years) and were onmore immunosuppressants/DMARDs. The patients in the >4 NLR group had significantly elevated total leucocyte count(TLC) 5560 (3360-11480) cells/mm3 and CRP 4.46 (0.3-48.2) mg/L and more patients were on steroid therapy. The >2-4NLR group had moderate inverse correlation with SLEDAI. NLR, ESR, CRP, and C3 did not show agreement with SLEDAI.The NLR was associated with CRP and steroid usage and could not discriminate disease severity.Conclusion: The relationship of the NLR with SLEDAI was not consistent. NLR was associated with CRP and steroid use.NLR as a marker of inflammation or as a predictor of SLE disease activity needs further investigation.

Central pontine myelinolysis after liver transplantation: case report / Mielinolisis Póntica Central post-trasplante hepático: Reporte de Caso

SUMMARY Central Pontine Myelinolysis (CPM) is a demyelinating disease with central pontine localization which presents in patients with chronic hepatic disease and hydroelectrolytic disturbances. In spite of pathophysiology still unknown, the patient liver transplanted has several risk factors to CPM. Its clinical manifestations are mainly motor and awareness alterations. Diagnosis requires brain images like magnetic resonance and it has a poor prognosis in critical care patients and it is very important to know about the possibility of presentation and to be aware about this pathology. The main objective to present this case report is sensitizing the medical community regarding the CPM in patients with liver transplant. The case report of a 40-year-old female patient who had previous history of biliary cirrhosis secondary to autoimmune hepatitis and liver transplantation was performed; subsequently developed alteration of awareness, hydroelectrolytic disturbances and abnormal findings in brain imaging. CPM is a neurological demyelinating disease with multifactorial origin, its treatment is supportive and prevention is the main goal in patients with risk factors like patient after liver transplantation.

RESUMEN La Mielinólisis Pontica Central es una enfermedad desmielinizante con localización pontina central que se presenta especialmente en pacientes con enfermedad hepática crónica y alteraciones hidroelectrolíticas. A pesar de la fisiopatología aún desconocida, el paciente con trasplante hepático tiene varios factores de riesgo para su desarrollo. Sus manifestaciones clínicas son principalmente alteraciones motoras y de conciencia. El diagnóstico requiere imágenes cerebrales como la resonancia magnética y se asocia a mal pronóstico. En los pacientes de cuidados críticos y es muy importante conocer la posibilidad de presentación y tener conciencia de esta patología. El objetivo principal de este reporte de caso es sensibilizar a la comunidad médica respecto en pacientes posterior a trasplante hepático. Se realizó el reporte de una paciente de 40 años de edad con antecedente de cirrosis biliar secundaria a hepatitis autoinmune y trasplante de hígado; posteriormente desarrolló alteración de la conciencia, alteraciones hidroelectrolíticas y hallazgos anormales en la imagen cerebral. La Mielinolísis Póntica es una enfermedad desmielinizante neurológica de origen multifactorial, su tratamiento es de apoyo y la prevención es el objetivo principal en pacientes con factores de riesgo como el paciente tras trasplante hepático.

Lupus eritematoso sistémico masculino / Systemic male lupus erythematosus

El Lupus Eritematosos Sistémico es el paradigma del síndrome autoinmune sistémico, cuya etiología está lejos de ser aclarada, aunque el conocimiento de su patogenia ha avanzado en estos últimos años inexorablemente, como el de los secretos más ocultos del funcionamiento del sistema autoinmune. Es mucho más frecuente en mujeres (10:1) y suele presentarse en la adolescencia tardía y a los 50 años, también es más frecuente y grave en algunos grupos étnicos, en especial afroamericanos e hispanos; su carácter crónico, su gran variedad clínica, sus episodios de activaciones y remisiones, la presencia de numerosos anticuerpos y la respuesta al tratamiento inmunosupresor son muestra de su naturaleza autoinmune. Presentamos el caso de un paciente de sexo masculino, de 38 años de edad, de origen hispano, que debutó con serositis, y que respondió de forma adecuada al tratamiento inmunosupresor instaurado.

Systemic Lupus Erythematosus is the paradigm of systemic autoimmune syndrome, whose etiologyis farfrom being clarified, although the knowledge of its pathogenesis has inexorably advanced in recent years, such as the most hidden secrets of the functioning of the autoimmune system. It is much more common in women (10: 1) and usually occurs in late adolescence and at age 50, is also more frequent and severe in some ethnic groups, especially African American and Hispanic; its chronic nature, its great clinical variety, its episodes of activation and remission, the presence of numerous antibodies and the response to immunosuppressive treatment are indicative of its autoimmune nature. We present the case of a 38-year-old male patient of Hispanic origin, who debuted with serositis, and who responded adequately to the immunosuppressive treatment instituted.

Tratamento das manifestações orais da doença enxerto contra hospedeiro crônica: revisão sistemática da literatura / Treatment of oral manifestations of chronic graft versus host disease: systematic literature review

Objetivo: Sintetizar as principais modalidades terapêuticas utilizadas no tratamento das manifestações orais da doença enxerto contra hospedeiro crônica (DECHc), através de uma revisão sistemática da literatura. Material e Métodos: Foi realizada uma busca nas bases de dados Cochrane Library,Dentistry & Oral Sciences e Pubmed, com descritores para o tratamento da DECHc com envolvimento oral, entre os meses de Junho e Outubro de 2015. Definiu-se uma metodologia de seleção a partir da leitura dos títulos, resumos e artigos disponíveis. Resultados: Foram encontradas 32 publicações, que apresentaram um total de 13 fármacos imunossupressores, sendo 07 dos quais foram empregados por meio de terapia local e 06 via sistêmica. Dois artigos tratavam de terapias auxiliares para o alívio da hipossalivação. Conclusão: As medicações encontradas podem servir de parâmetros para futuros fármacos a serem empregados na prática clínica. O tratamento da DECHc oral ainda apresenta eficácia limitada, todavia, sugere-se o uso de medicação tópica para reduzir efeitos sistêmicos. São necessários estudos prospectivos, randomizados e controlados para comprovar a eficácia de certas medicações.

Objective: To summarize the main therapeutic modalities used in the treatment of oral manifestations of graft versus host disease (GVHD), through a systematic review of the literature. Material and Methods: A search was conducted in the databases Cochrane Library, Dentistry & Oral Sciences and Pubmed, with descriptors for the treatment of chronic GVHD with oral involvement, between June and October 2015. The methodology was defined from reading the titles, abstracts and articles available. Results: Founded 32 publications that presented a total of 13 immunosuppressant drugs, and 07 which were employed by local therapy and systemically 06. Two articles treated auxiliary therapies to relieve hyposalivation. Conclusion: Drugs found may serve as parameters for future drugs to be used in clinical practice. The treatment of oral GVHD still has limited effectiveness, however, suggests the use of topical medications to reduce systemic effects. Prospective studies are needed, randomized controlled trials to prove the effectiveness of these medications.

Hiperuricemia, enfermedad renal crónica y trasplante renal (parte II) / Hyperuricemia, chronic renal disease and renal transplant (part II)

La hiperuricemia (HU) en el trasplante renal (TR) ha sido definida igual que en la población general en las Guías KDIGO como valores por encima de 6 mg/dl en mujeres y 7 mg/dl en hombres. La incidencia de HU en algunas poblaciones es de 28%,(1) alcanzando el 80% en la era Ciclosporina (CSA).(2) La HU se observa precozmente luego del TR, los factores de riesgo asociados con su desarrollo incluyen: edad avanzada al momento del TR; historia de gota o HU pre-existente; obesidad; presencia de síndrome metabólico (SM); deterioro de la función del injerto; uso de inmunosupresores, principalmente ciclosporina (CSA); uso de diuréticos

Hyperuricemia (HU) in renal transplant (RT) has been defined, like general population, with KDIGO Guides, as over 6 mg/dl values in women and 7 mg/dl in men. HU incidence in some populations are 28%, reaching 80% in Cyclosporine era (CSA). HU is early observed after RT, risk factors associated with its development include: advanced age at the time of RT; gout history or pre-existing HU; obesity; metabolic syndrome presence (MS); graft function deterioration; use of Inmunosuppression drugs, mainly cyclosporine (CSA); use of diuretics

Signaling pathway inhibitors of calcineurin-nuclear factor of activated T cells:research progress / 中国药理学与毒理学杂志

Calcineurin (CaN) serves as a key enzyme in human immune regulation. The most important target of this enzyme is the transcription factors of nuclear factors of activated T cells (NFATc). The discovery of the immunosuppressive function of CaN inhibitors (CNIs),ciclosporin A (CsA) and tacrolimus (FK506),has helped overcome the immune rejection of organ transplantion and changed organ transplantion fundamentally. Both of these drugs are still widely used in clinical and basic research,but their therapeutic effects are limited by their serious side effects,including renal tox?icity and neurotoxicity. Therefore,the development of new CNIs with higher specificity and fewer side effects in the clinic is a focus of research. In this paper,the newly discovered and synthesized CNIs in recent decades,including the CsA and FK506 derivatives,direct inhibitors of CaN,as well as the inhibitors that specifically interfere with CaN-NFATc interaction,were summarized.

Suppressive effect of a novel benzothiazole derivative BD759 on T cell proliferation / 中国药学杂志

OBJECTIVE To determine the immunosuppressive activity of a novel benzothiazole derivative BD759 on T cell proliferation and its potential mode of action. METHODS: T cell proliferation, CD25 expression and cell cycle distribution were measured by flow cytometer. Cytokine levels, including IL-2, IL-4, IL-6, IL-10, IL-17A and IFN-γ, were determined by ELISA. RESULTS: BD759 significantly inhibited human T cell proliferation, stimulated either by anti-CD3/anti-CD28 monoclonal antibodies or by an al-loantigen, in a dose-dependent manner with IC50 values of (3.5 ± 0.7) and (3.3 ± 0.9) μmol · L-1, respectively. No obvious cytotoxic effects of BD759 were observed on human resting naive T cells and peripheral blood mononuclear cells in our experimental conditions. Furthermore, BD759 did not inhibit CD25 expression or IL-2, IL-4 and IL-10 secretion, but inhibited IL-6, IL-17A and IFN-γ production and induced cell cycle arrest at the G0/G1 phase in activated T cells. CONCLUSION: These data indicate that BD759 has no effect on T cell activation, but induces T cell cycling arrest at G0/G1 phase. BD759 also inhibits the secretion of inflammatory cytokines, such as IL-6, IL-17A and IFN-gamma. Thus, BD759 has the potential to be used as a lead compound for the design and development of new immunosuppressants for treating autoimmune diseases and preventing graft rejection.

Sequence and timing of immunosuppressants-thymectomy combination therapy in patients with myasthenia gravis / 中华胸心血管外科杂志

Objective Following the prognosis of patients with myasthenia gravis(MG) who received both immunosuppressants and thymectomy, to determine whether the sequence and timing of treatment have influences on outcomes of MG and also to select significant predictors of prognosis.Methods It was a retrospective study which reviewed 105 patients with MG who underwent thymectomy and immunosuppressants between Januay, 2000 and December, 2013.All patients were divided into two subgroups based on their order of treatment.Thirty two patients were called as immunosuppressants-first group, and 73 patients were called as thymectomy-first group.The ratios of ideal status of two groups were compared with use of the ChiSquare test.Predictors of ideal status were analyzed by logistic regression.Results (1) After 1 year, 2 years and 5 years of intervention, the proportion of ideal status was significantly higher in the thymectomy-first group than that in the immunosuppressants-first group[(33％-50％) vs.(10％-20％), P ＜0.05].For oMG patients, after 1 year, 2 years and 5 years of treatment, thymectomy-first group had a higher ideal status rates than immunosuppressants-first group[(25％-50％) vs.(11％-18％)].But no significant difference was noted in the rates of ideal status at each follow-up time point.(2)Multivariate logistic regression analysis showed that sequence of treatment (OR =0.154, 95 ％ CI: 0.046-0.518, P =0.002) and symptom-thymectomy interval(OR =0.903,95 ％ CI: 0.835-0.976, P =0.010) were the significant predictors of ideal status.Conclusion Thymectomy prior to immunosuppressants is associated with relatively good prognosis.It is suggested that patients with gMG, or adult patients with oMG who are resistant to drug, will be better to undergo thymecotomy as early as possible.