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1.
Einstein (Säo Paulo) ; 18: eAO5132, 2020. tab, graf
Article in English | LILACS | ID: biblio-1056070

ABSTRACT

ABSTRACT Objective To evaluate the performance of enzyme-linked immunosorbent assay and indirect immunofluorescence methods for the detection of antineutrophil cytoplasmic antibodies in a routine clinical laboratory setting. Methods A total of 227 samples were tested by indirect immunofluorescence and enzyme-linked immunosorbent assay with antigen specificity for antiproteinase 3 and antimyeloperoxidase. The proportions of positive samples were compared by McNemar hypotheses and agreement was described by Cohen's Kappa coefficient. Results The agreement of the tests was 96.5%, and the Kappa coefficient obtained was 0.70 (95%CI: 0.50-0.90; p<0.001). Considering indirect immunofluorescence as the gold standard, the sensitivity of the enzyme-linked immunosorbent assay was 0.62 and the specificity was 0.99, with diagnostic accuracy in 96% of cases. Some samples were negative in enzyme-linked immunosorbent assay and positive in indirect immunofluorescence. This situation occurred in all immunofluorescence patterns, but particularly in atypical patterns. Two samples with antiproteinase 3 positivity were considered negative in indirect immunofluorescence. Conclusion The enzyme-linked immunosorbent assay had high specificity but lower sensitivity. The performance of indirect immunofluorescence increases diagnostic sensitivity, while the search for antiproteinase 3 by enzyme-linked immunosorbent assay may also add diagnostic power.


RESUMO Objetivo Avaliar o desempenho das metodologias de ensaio imunoenzimático e imunofluorescência indireta para a detecção de anticorpos anticitoplasma de neutrófilos em um contexto de laboratório clínico de rotina. Métodos Foram testadas 227 amostras pelas metodologias de imunofluorescência indireta e ensaio imunoenzimático com especificidades para anticorpos antiproteinase-3 e antimieloperoxidase. As proporções de amostras positivas foram comparadas por hipóteses de McNemar, e a concordância foi descrita pelo coeficiente Kappa de Cohen. Resultados A concordância dos testes foi 96,5%, e o coeficiente Kappa obtido foi 0,70 (IC95%: 0,50-0,90; p<0,001). Utilizando a imunofluorescência indireta como padrão-ouro, a sensibilidade do ensaio imunoenzimático foi de 0,62 e a especificidade, 0,99, com acurácia diagnóstica em 96% dos casos. Algumas amostras apresentaram resultados negativos por ensaio imunoenzimático e positivos por imunofluorescência. Isso ocorreu em amostras com vários padrões de fluorescência, mas particularmente nos casos com padrões atípicos. Duas amostras com positividade antiproteinase 3 foram consideradas negativas por imunofluorescência. Conclusão Os métodos de ensaio imunoenzimático tiveram alta especificidade, mas sensibilidade inferior. A realização da imunofluorescência indireta aumenta a sensibilidade diagnóstica, ao mesmo tempo que a pesquisa de antiproteinase 3 por ensaio imunoenzimático também pode agregar poder diagnóstico.


Subject(s)
Humans , Enzyme-Linked Immunosorbent Assay/methods , Fluorescent Antibody Technique, Indirect/methods , Antibodies, Antineutrophil Cytoplasmic/blood , Reference Standards , Reference Values , Autoimmune Diseases/diagnosis , Autoimmune Diseases/immunology , Autoimmune Diseases/blood , Predictive Value of Tests , Reproducibility of Results
3.
Gac. méd. Méx ; 155(1): 63-71, Jan.-Feb. 2019. tab, graf
Article in English, Spanish | LILACS | ID: biblio-1286461

ABSTRACT

Resumen Los microRNA (miRNA) son pequeños RNA no codificantes de aproximadamente 17 a 24 nucleótidos de longitud, los cuales se unen complementaria y principalmente en las regiones 3' UTR (región no traducida) de diversos RNA mensajeros (mRNA, messenger RNA). Su función general es regular negativamente la expresión génica a nivel postranscripcional, inhibiendo la traducción. Perfiles de expresión de miRNA alterados han sido identificados en diferentes líquidos, células y tejidos humanos afectados con diversas enfermedades autoinmunes y algunos se han propuestos potencialmente como biomarcadores de diagnóstico, pronóstico, actividad, etcétera, en estas patologías. Adicionalmente, variantes comunes del genoma humano, denominados polimorfismos de un solo nucleótido (SNP, single nucleotide polymorphisms) localizados en genes de miRNA han sido asociados con susceptibilidad, gravedad, y actividad en estas enfermedades. El objetivo de esta revisión es describir la biogénesis de los miRNA, su función, así como los perfiles de expresión y SNP en genes de miRNA asociados con diversas enfermedades autoinmunes, incluyendo tiroiditis autoinmune (tiroiditis de Hashimoto y enfermedad de Graves), lupus eritematoso sistémico, artritis reumatoide y síndrome de Sjögren primario.


Abstract MicroRNAs (miRNAs) are small non-coding RNAs of approximately 17-24 nucleotides in length, which complementarily and mainly bind in 3' UTR (untranslated region) regions of different messenger RNAs (mRNAs). Their general function is to negatively regulate gene expression at the posttranscriptional level, thus inhibiting translation. miRNA abnormal expression profiles of have been found in different human fluids, cells and tissues affected by different autoimmune diseases, and some of them have been proposed as potential biomarkers of diagnosis, prognosis, activity etc. in these pathologies. In addition, common variants of the human genome, called single-nucleotide polymorphisms (SNPs), located within miRNA genes, have been associated with susceptibility, severity and activity in these diseases. The purpose of this review is to describe miRNA biogenesis and function, as well as the expression profiles and SNPs in miRNA genes that are associated with different autoimmune diseases, including autoimmune thyroiditis (HashimotoMs thyroiditis and Gravess disease), systemic lupus erythematosus, rheumatoid arthritis and primary Sjögren's syndrome.


Subject(s)
Humans , Autoimmune Diseases/genetics , Gene Expression Regulation , MicroRNAs/genetics , Autoimmune Diseases/physiopathology , Autoimmune Diseases/immunology , Severity of Illness Index , Polymorphism, Single Nucleotide
4.
Rev. chil. reumatol ; 35(2): 44-54, 2019. ilus
Article in Spanish | LILACS | ID: biblio-1281801

ABSTRACT

Miopatía Necrotizante Autoinmune (MNA) fue reconocida como nuevo sub-grupo de miositis luego de observar en biopsias musculares la presencia de necro-sis con escaso o ausente infiltrado inflamatorio, sumado a la expresión de dos an-ticuerpos específicos de miositis (Anticuerpo anti Partícula de Reconocimiento de Señal, anti-SRP; y Anticuerpo anti Hidroxi-3-metilglutaril-CoA reductasa, anti-HM-GCR), ambos fuertemente asociados al hallazgo histológico descrito y a fenotipos clínicos característicos a cada anticuerpo, los cuales comparten importantes simi-litudes representadas por severa debilidad muscular proximal, gran elevación de creatinkinasa (CK), escasa manifestación de síntomas y signos extramusculares, y resistencia al uso de inmunosupresión habitual. Si bien en primera instancia los criterios de clasificación propuestos estaban basados en la histología, la obser-vación de necrosis en otros subgrupos de miositis, sumado a la homogeneidad del comportamiento clínico de pacientes que expresaban anticuerpos anti-SRP o anti-HMGCR independiente de la histología presentada, llevó en el año 2016 al Grupo de Trabajo del Centro Europeo Neuromuscular (ENMC) a establecer crite-rios diagnósticos de MNA basados en el comportamiento clínico (debilidad mus-cular proximal con CK total elevada) más la presencia del anticuerpo respectivo (anti-SRP o anti-HMGCR), reservando la necesidad de realizar biopsia muscular en el caso que la serología resulte negativa, siendo así reconocidas tres entidades distintas de MNA: Miopatía anti-SRP, Miopatía anti-HMGCR y Miopatía Necroti-zante seronegativa. La presente revisión expresa el actual conocimiento de MNA y sus subtipos, refiriéndose a aspectos históricos, clínicos, histológicos, inmuno-patológicos, y de pronóstico y tratamiento.


Necrotizing autoinmune myopathy (NAM) was recognized as a new sub-group of myositis after the observation of necrosis with mild or absent inflam-matory infiltrates in muscle biopsies, in addition of expression of two specific myositis antibodies (antiSRP and antiHMGCR), which are strongly associated to the mentioned hystologic findings, with different clinical phenotypes depending on the presence of each antibody, but sharing some features like severe proximal muscle weakness, significant elevation of creatin phosphokinase (CK), mild ex-tramuscular involvement and resistance to commonly used immunosupressants. The first proposed approach to classification criteria was hystology-based, none-theless the observation of necrosis in some other types of myositis and the homo-geneity of clinical features in patients expressing antiSRP or antiHMGCR despite the hystologic findings led to a new classification scheme leaded by the European Neuromuscular Center in 2016, which recognizes thre different clinical entities of NAM, based on the antibody expression plus the presence of proximal muscle weakness, relying hystology to a secondary place thus eliminating the need for immediate biopsy to stablish a diagnosis: those are antiSRP myopathy, antiHMG-CR myopathy and seronegative necrotizing myopathy, being the last one the only needing muscle biopsy. The present review shows the actual knowledge about NAM and its subtypes, referring to hystoric, clinical, hystologic, immunopatholog-ic, prognostic and therapeutic issues.


Subject(s)
Humans , Autoimmune Diseases/pathology , Myositis/pathology , Autoimmune Diseases/immunology , Muscular Diseases , Myositis/diagnosis , Myositis/physiopathology , Myositis/therapy , Myositis/epidemiology , Necrosis/immunology , Necrosis/pathology
5.
Int. j. morphol ; 36(4): 1509-1513, Dec. 2018. graf
Article in English | LILACS | ID: biblio-975729

ABSTRACT

IgG4-related disease is a systemic, multifocal, immune-mediated disorder that can affect multiple organs and may present as a tumor, with rare cases described in the maxillofacial region. A female patient, 53 years old, presenting tumor-like mass in the right mandibular region. Magnetic resonance imaging suggested well circumscribed nodular lesion adjacent to the branch / body of the mandible, extending posteriorly to the masseter muscle. During the surgical procedure of excision, a lesion was observed adhering to the right masseter muscle, but it was possible to remove it completely. Histopathological and immunehistochemical analysis suggested diagnosis of IgG4-related disease, furthermore, IgG4 serum count was increased. Actually, the patient continues on periodical followups in our service and by other specialties. Can be concluded that precise diagnosis of this pathology depends on many factors, being challenging and the treatment involves multidisciplinary evaluation due to the possibility of involvement of several other organs.


La enfermedad relacionada con IgG4 es una condición sistémica, multifocal, mediada por una alteración de la respuesta inmune que puede afectar diferentes órganos o puede presentarse como un tumor, raramente descrito en el área maxilofacial. Se describe el caso de una paciente de sexo femenino de 53 años de edad, presentando una masa tumoral en el ángulo mandibular derecho. La resonancia magnética sugirió un área nodular bien delimitada adyacente al cuerpo mandibular y extendida posteriormente hasta el musculo masetero. Durante la escisión quirúrgica, la lesión se presentaba adherida al musculo de forma lateral siendo posible el retiro total de la lesión. El estudio histopatológico e inmunohistoquimico determinó el diagnóstico de enfermedad relacionada con IgG4 presentando un conteo de igG4 aumentado. Actualmente, la paciente continua con seguimiento por la especialidad. Se puede concluir que el diagnóstico preciso de esta patología depende de algunos factores; el tratamiento debe ser multidsciplinario debido a la inclusión de diferentes órganos en la enfermedad.


Subject(s)
Humans , Female , Middle Aged , Autoimmune Diseases/pathology , Immunoglobulin G , Jaw Neoplasms/pathology , Autoimmune Diseases/immunology , Autoimmune Diseases/diagnostic imaging , Immunohistochemistry , Magnetic Resonance Imaging , Jaw Neoplasms/immunology , Jaw Neoplasms/diagnostic imaging
6.
Arch. endocrinol. metab. (Online) ; 62(5): 566-570, Oct. 2018. tab, graf
Article in English | LILACS | ID: biblio-983788

ABSTRACT

SUMMARY Insulin autoimmune syndrome (IAS, Hirata's disease) is a rare hypoglycemic disorder characterized by spontaneous hypoglycemia associated with extremely high circulating insulin levels and positive anti-insulin antibody results. Thus far, most cases have been reported in Asian countries, notably Japan, with few cases reported in western countries. As a possible cause, it is associated with the use of drugs containing sulfhydryl radicals, such as captopril. This report refers to a 63-year-old female Brazilian patient with a history of postprandial hypoglycemia. After extensive investigation and exclusion of other causes, her hyperinsulinemic hypoglycemia was considered to have likely been induced by captopril. Most cases of IAS are self-limiting. However, dietary management, corticosteroids, plasmapheresis, and rituximab have already been used to treat patients with IAS. In our case, after discontinuation of captopril, an initial decrease in insulin autoantibody levels was observed followed by improvement in episodes of hypoglycemia. Although it is a rare disease, IAS should be considered in the differential diagnosis of endogenous hyperinsulinemic hypoglycemia. Patients with suspected IAS must be screened for autoimmunity-related drugs for insulin. Initial clinical suspicion of IAS can avoid unnecessary costs associated with imaging examinations and/or invasive surgical procedures.


Subject(s)
Humans , Female , Middle Aged , Autoimmune Diseases/chemically induced , Captopril/adverse effects , Hypoglycemia/chemically induced , Hypoglycemia/immunology , Insulin Antibodies/drug effects , Antihypertensive Agents/adverse effects , Autoimmune Diseases/ethnology , Autoimmune Diseases/immunology , Syndrome , Blood Glucose/analysis , Brazil , Hypoglycemia/ethnology , Insulin Antibodies/immunology
7.
Rev. Assoc. Med. Bras. (1992) ; 63(12): 1090-1099, Dec. 2017. graf
Article in English | LILACS | ID: biblio-896334

ABSTRACT

Summary Previous studies have demonstrated the expression of the CD25 marker on the surface of naturally occurring T cells (Tregs) of mice, which have a self-reactive cellular profile. Recently, expression of other markers that aid in the identification of these cells has been detected in lymphocyte subtypes of individuals suffering of autoimmune and idiopathic diseases, including: CD25, CTLA-4 (cytotoxic T-lymphocyte antigen 4), HLA-DR (human leukocyte antigen) and Interleukin 10 (IL-10), opening new perspectives for a better understanding of an association between such receptors present on the cell surface and the prognosis of autoimmune diseases. The role of these molecules has already been described in the literature for the modulation of the inflammatory response in infectious and parasitic diseases. Thus, the function, phenotype and frequency of expression of the a-chain receptor of IL-2 (CD25) and IL-10 in lymphocyte subtypes were investigated. Murine models have been used to demonstrate a possible correlation between the expression of the CD25 marker (on the surface of CD4 lymphocytes) and the control of self-tolerance mechanisms. These studies provided support for the presentation of a review of the role of cells expressing IL-2, IL-10, HLA-DR and CTLA-4 receptors in the monitoring of immunosuppression in diseases classified as autoimmune, providing perspectives for understanding peripheral regulation mechanisms and the pathophysiology of these diseases in humans. In addition, a therapeutic approach based on the manipulation of the phenotype of these cells and ways of scintigraphically monitoring the manifestations of these diseases by labeling their receptors is discussed as a perspective. In this paper, we have included the description of experiments in ex vivo regulation of IL-10 and synthesis of thio-sugars and poly-sugars to produce radiopharmaceuticals for monitoring inflammation. These experiments may yield benefits for the treatment and prognosis of autoimmune diseases.


Resumo Estudos anteriores já haviam demonstrado a expressão do marcador CD25 na superfície de células T de ocorrência natural (Tregs) de camundongos, que apresentam perfil celular autorreativo. Recentemente, foi detectada, em subtipos de linfócitos de indivíduos acometidos por doenças autoimunes e de causa idiopática, a expressão de outros marcadores, que auxiliam na identificação dessas células, entre os quais: CD25, CTLA-4 (cytotoxic T-lymphocyte antigen 4), HLA-DR (human leucocyte antigen) e Interleucina 10 (IL-10), abrindo novas perspectivas para a melhor compreensão de uma associação entre esses receptores presentes na superfície celular e o prognóstico de doenças autoimunes. O papel dessas moléculas já havia sido descrito na literatura na modulação da resposta inflamatória em doenças infectoparasitárias. Dessa forma, foram investigados a função, o fenótipo e a frequência de expressão, do receptor de cadeia a da IL-2 (CD25) e de IL-10 em subtipos de linfócitos. O modelo murino tem sido utilizado para demonstrar uma possível correlação entre a expressão do marcador CD25 (na superfície de linfócitos CD4) e o controle dos mecanismos de autotolerância. Essas pesquisas forneceram suporte para apresentação de uma revisão sobre o papel das células que expressam os receptores de IL-2, IL-10, HLA-DR e CTLA-4 no monitoramento da imunossupressão, em doenças de classificação autoimune, abrindo perspectivas para o entendimento dos mecanismos de regulação periférica e sobre a fisiopatologia dessas doenças no ser humano. Além disso, é discutida como perspectiva uma abordagem terapêutica fundamentada na manipulação do fenótipo dessas células, bem como de modos de monitoramento cintilográfico das manifestações dessas doenças, por meio da marcação de seus receptores. Nestes, foram incluídas descrições das experiências em regulação ex-vivo de IL-10; de síntese de tioaçúcares e de poliaçúcares para produção de radiofármacos para monitoramento de inflamações. Essas experiências podem trazer benefícios na terapia e no prognóstico de doenças autoimunes.


Subject(s)
Humans , Animals , Autoimmune Diseases/diagnostic imaging , Autoimmunity/physiology , Interleukin-10/physiology , T-Lymphocytes, Regulatory/physiology , Prognosis , Autoimmune Diseases/immunology , Autoimmune Diseases/therapy , HLA-DR Antigens , Radionuclide Imaging , CD4 Antigens/immunology , Interleukin-10/immunology , Models, Animal , Interleukin-2 Receptor alpha Subunit/immunology , CTLA-4 Antigen , Immune Tolerance , Mice
8.
An. bras. dermatol ; 92(1): 30-33, Jan.-Feb. 2017. tab
Article in English | LILACS | ID: biblio-838023

ABSTRACT

Abstract: BACKGROUND: Drug reaction with eosinophilia and systemic symptoms is a severe adverse drug reaction, with a reported mortality of 10%. Long-term outcomes involve organic failure and autoimmune diseases in some populations. OBJECTIVE: To evaluate the clinical prognosis of patients with drug reaction with eosinophilia and systemic symptoms. METHODS: We conducted a retrospective review at a referral hospital in Mexico City in a period of 22 years (1992-2013), looking up for records with diagnosis of DRESS according to RegiSCAR criteria. Clinical characteristics, organ failures, culprit drugs, treatment, and short and long-term sequelae were analyzed. RESULTS: We found 11 patients with diagnosis of drug reaction with eosinophilia and systemic symptoms syndrome, 7 female and 4 male, with a median age of 22 years-old; 9 had maculopapular rash and 2 were erythrodermic. Affected organs were liver (8/11), kidney (6/11) and hematologic disorders (8/11). The most common culprit drugs were antiepileptic (63%). Systemic corticosteroids were given to 8 patients, being pyelonephritis (1/8) and pneumonia (2/8) the adverse events of this therapy. Long-term sequelae were 1 patient with renal failure, 1 patient with chronic anemia; and 2 patients developed autoimmune diseases (one autoimmune thyroid disease and another one with autoimmune thyroid disease and autoimmune hemolytic anemia). Study limitations: The retrospective nature of the study and the limited number of patients with drug reaction with eosinophilia and systemic symptoms. CONCLUSIONS: Drug reaction with eosinophilia and systemic symptoms syndrome has been linked to the development of chronic organ failure. We found two young patients who developed autoimmune diseases in the short term. Patients with drug reaction with eosinophilia and systemic symptoms should have a long-term monitoring for signs or symptoms suggestive of an autoimmune disease.


Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Young Adult , Autoimmune Diseases/etiology , Drug Hypersensitivity Syndrome/etiology , Prognosis , Autoimmune Diseases/immunology , Retrospective Studies , Drug Hypersensitivity Syndrome/immunology , Mexico
9.
Medicina (B.Aires) ; 76(3): 129-134, June 2016. ilus, tab
Article in English | LILACS | ID: biblio-841559

ABSTRACT

The idiopathic inflammatory myopathies(IIM) are a heterogeneous group of diseases of the skeletal muscle. On the basis of clinical, serologic and histological differences, they are classified in dermatomyositis (DM), polymyositis (PM), inclusion body myositis and immunomediated necrotizing myopathy. Autoantibodies directed against nuclear and cytoplasmic antigens are present with variable frequencies among studies. Myositis-specific antibodies (MSAs) are useful in IIM because they contribute to the diagnosis, help to identify different clinical subsets, and have prognostic value. This study aimed to explore the frequency of autoantibodies, especially MSAs, and their relationship with clinical features in adult patients with DM, PM and overlap syndrome. Medical records were reviewed. Myositis-associated antibodies (non-specific) and MSAs (anti Jo-1, PL-7, PL-12, Mi-2 and SRP) were measured using commercial kits. Twelve patients had MSAs, an overall frequency similar to those of international series, but PL-12 and Mi-2 were more frequent than Jo-1, which is the most frequently observed elsewhere. All five patients with Mi-2 had classical DM with a favorable response to treatment. Interstitial pneumonia (n: 4) and/or treatment-refractory disease (n: 3) were found in the presence of anti-PL-12, alone or associated with anti-SRP and/or Jo-1. In conclusion, the coexistence of AEM, a rare finding, was found in three patients. The presence of MSAs aided to the diagnosis of IIM, in particular in those patients without available or conclusive biopsy results.


Las miopatías inflamatorias idiopáticas (MII) comprenden un grupo heterogéneo de enfermedades adquiridas del músculo esquelético. Según sus características clínicas, serológicas e histológicas se las clasifica en dermatomiositis (DM), polimiositis (PM), miopatía necrotizante autoinmune y miositis por cuerpos de inclusión. Los anticuerpos específicos de miositis (AEMs) contribuyen al diagnóstico, permiten distinguir formas clínicas y tienen valor pronóstico. Con el objetivo de explorar la frecuencia de autoanticuerpos, en particular AEMs, y su relación con las características clínicas de las MII del adulto, se revisaron las historias clínicas de 25 pacientes con DM, PM y síndromes de superposición, asistidos en nuestro centro entre 1999 y 2013. La presencia de autoanticuerpos asociados a miositis (no específicos) y AEMs (anti Jo-1, PL-7, PL-12, Mi-2, SRP) se investigó utilizando kits comerciales. Doce pacientes presentaron AEMs, frecuencia global similar a la encontrada en series internacionales, pero a diferencia de lo observado en otros países, anti-PL-12 y anti-Mi-2 fueron más frecuentes que anti-Jo-1. Los cinco pacientes con anti-Mi-2 tuvieron DM clásica y buena evolución clínica. Anti-PL-12, ya sea solo o asociado a anti-SRP y/o anti-Jo-1, estuvo presente en pacientes con neumonía intersticial (n:4) y/o enfermedad refractaria al tratamiento (n: 3). En conclusión, la coexistencia de AEM, hallazgo raro, se encontró en tres pacientes. La presencia de AEMSs contribuyó al diagnóstico de MII, en particular en aquellos casos sin resultados concluyentes de biopsia de músculo.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Autoantibodies/analysis , Autoimmune Diseases/immunology , Polymyositis/immunology , Dermatomyositis/immunology , Argentina , Reference Values , Autoimmune Diseases/diagnosis , Autoimmune Diseases/pathology , Biopsy , Muscle, Skeletal/pathology , Dermatomyositis/diagnosis , Dermatomyositis/pathology
10.
Braz. j. med. biol. res ; 49(5): e5209, 2016. tab, graf
Article in English | LILACS | ID: biblio-951671

ABSTRACT

The traditional concept that effector T helper (Th) responses are mediated by Th1/Th2 cell subtypes has been broadened by the recent demonstration of two new effector T helper cells, the IL-17 producing cells (Th17) and the follicular helper T cells (Tfh). These new subsets have many features in common, such as the ability to produce IL-21 and to express the IL-23 receptor (IL23R), the inducible co-stimulatory molecule ICOS, and the transcription factor c-Maf, all of them essential for expansion and establishment of the final pool of both subsets. Tfh cells differ from Th17 by their ability to home to B cell areas in secondary lymphoid tissue through interactions mediated by the chemokine receptor CXCR5 and its ligand CXCL13. These CXCR5+ CD4+ T cells are considered an effector T cell type specialized in B cell help, with a transcriptional profile distinct from Th1 and Th2 cells. The role of Tfh cells and its primary product, IL-21, on B-cell activation and differentiation is essential for humoral immunity against infectious agents. However, when deregulated, Tfh cells could represent an important mechanism contributing to exacerbated humoral response and autoantibody production in autoimmune diseases. This review highlights the importance of Tfh cells by focusing on their biology and differentiation processes in the context of normal immune response to infectious microorganisms and their role in the pathogenesis of autoimmune diseases.


Subject(s)
Humans , Autoimmune Diseases/immunology , Autoimmunity/immunology , T-Lymphocytes, Helper-Inducer/immunology , B-Lymphocytes/immunology , Lymphocyte Activation/immunology , CD4-Positive T-Lymphocytes/immunology , Signal Transduction , Cell Differentiation , Interleukins/immunology , Th2 Cells/immunology , Interleukin-17/immunology , Th17 Cells/immunology
11.
Rev. chil. dermatol ; 32(1): 15-21, 2016. graf
Article in Spanish | LILACS | ID: biblio-911523

ABSTRACT

Las enfermedades autoinmunes son desórdenes heterogéneos que pueden comprometer distintos órganos. Su frecuencia es baja, se estima que 3 de cada 1.000 niños cursan con alguna condición reumatológica. Las patologías reumatológicas más comunes en la edad pediátrica son la artritis idiopática juvenil (AIJ) seguida por el lupus eritematoso sistémico (LES), dermatomiositis juvenil (DMJ), vasculitis primarias y la esclerodermia. Materiales y Métodos: Se efectuó un estudio descriptivo retrospectivo de pacientes pediátricos atendidos en el servicio de inmunología del Hospital Roberto del Rio entre los años 1990 y 2011. Se pesquisaron un total de 102 pacientes, con diagnósticos de AIJ, LES y DMJ. Se diseñó una ficha de protocolo, con los datos: edad, sexo, antecedentes familiares, manifestaciones cutáneas al diagnóstico y a lo largo de la evolución. Para el análisis estadístico de variables, se utilizó el programa STATA 8.0. Resultados: El 45,45% de los pacientes con AIJ presentó lesiones cutáneas, sin embargo, sólo un 20% de ellas, relacionadas a esta enfermedad. El 91,7% de los pacientes con LES presentó manifestaciones cutáneas, siendo la vasculitis cutánea y el eritema malar, las más frecuentes. En los pacientes con DMJ, el eritema heliotropo y pápulas de Gottron fueron las manifestaciones cutáneas más comunes. Conclusión: Los hallazgos cutáneos cobran un rol muy importante en el diagnostico enfermedades autoinmunes. Estos datos demuestran la importancia de un examen dermatólogico exhaustivo para su diagnóstico precoz y evitar sus complicaciones.


Background: Autoimmune diseases are disorders that can compromise different organs. Its frequency is low, it is estimated that 3 out of every 1,000 children are affected with any rheumatologic condition. The most common rheumatic diseases in children are juvenile idiopathic arthritis (JIA) followed by systemic lupus erythematosus (SLE), juvenile dermatomyositis (DMJ), primary vasculitis and esclerodermia.2 Materials and Methods: A retrospective study was conducted in pediatric patients seen in the Department of Immunology from the Roberto del Rio Hospital between 1990 and 2011. Records of 105 patients with the diagnosis of juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE) and juvenile dermatomyositis (JDM) have been included. We designed a protocol file with the given data: Age, sex, family history, skin manifestations at the diagnosis and throughout the evolution. The program STATA 8.0 was used for statistical analysis of variable. Results: 45.45% of JIA patients had some type of skin lesions, however, only 20% of them related to this disease. 91.7% of SLE patients presented cutaneous manifestations, the most common being cutaneous vasculitis and malar erythema. In patients with JDM, heliotrope erythema and papules Gottron were the most common skin manifestations. Discussion: Cutaneous manifestations have a very important role in autoimmune diseases. While the diagnosis and management of these diseases require a multidisciplinary team, these data demonstrate the importance of an exhaustive physical examination for early diagnosis and thereby reduce complications. Conclusions: This study highlights the importance of the dermatologist in an early diagnosis of rheumatic diseases.


Subject(s)
Humans , Male , Female , Child , Skin Diseases/epidemiology , Autoimmune Diseases/epidemiology , Skin Diseases/immunology , Autoimmune Diseases/immunology , Epidemiology, Descriptive , Retrospective Studies
12.
Yonsei Medical Journal ; : 527-531, 2016.
Article in English | WPRIM | ID: wpr-165377

ABSTRACT

Autoimmune retinopathy (AIR) is an immune-mediated retinopathy, resulting from an immunologic process caused by the aberrant recognition of retinal antigens as autoantigens. The diagnosis of AIR involves the detection of antiretinal antibodies with concurrent clinical and electrophysiological evidence of retinopathy. A 40-year-old patient presented with progressive loss of bilateral vision over several months. A fundus examination was unremarkable. Spectral domain optical coherence tomography revealed a blurred photoreceptor ellipsoid zone at the subfoveal region in both eyes with more prominent disruption in the left eye. Full-field electroretinography (ERG) showed relatively normal rod and cone responses in the right eye, and decreased photopic bwaves with minimal attenuation of a-waves in the left eye. Multifocal ERG demonstrated slightly reduced amplitude of the inner segment ring in the right eye and decreased amplitudes and delayed latencies of all modalities in the left eye. The patient was suspected to have AIR and it was supported by positive Western blots for 23-kDa protein, enolase (46-kDa), aldolase (40-kDa), 62-kDa and 78-kDa proteins and by immunohistochemical staining of human retinal bipolar and ganglion cells. Despite the immunosuppressive treatment, the destruction of the retinal photoreceptors progressed, and immunosuppressive interventions produced very little visual improvement. We report on what is, to the best of our knowledge, the very first case of serologically confirmed nonparaneoplastic AIR in Korea.


Subject(s)
Autoantibodies/blood , Autoantigens , Autoimmune Diseases/immunology , Electroretinography , Humans , Immunologic Factors , Paraneoplastic Syndromes/immunology , Paraneoplastic Syndromes, Ocular , Phosphopyruvate Hydratase , Recoverin , Republic of Korea , Retina/immunology , Retinal Diseases/immunology , Tomography, Optical Coherence
13.
Rev. méd. Chile ; 143(8): 1042-1049, ago. 2015. ilus
Article in Spanish | LILACS | ID: lil-762671

ABSTRACT

Type 1A diabetes (DM1A) is an autoimmune disease that comprises 10% of patients with diabetes mellitus. Its frequency is gradually increasing in countries like Mexico. Patients with DM1A commonly have hypothyroidism, Addison disease, celiac disease and less common diseases such as polyglandular syndrome. These diseases are related to susceptibility genes such as HLA, CTLA-4 and PTPN22, which induce central and peripheral immunologic tolerance. This review article emphasizes the importance of searching other autoimmune diseases in patients with DM1A, to improve their prognosis and quality of life.


Subject(s)
Animals , Humans , Autoimmune Diseases , Diabetes Mellitus, Type 1 , Addison Disease/immunology , Autoimmune Diseases/diagnosis , Autoimmune Diseases/genetics , Autoimmune Diseases/immunology , Celiac Disease/immunology , Diabetes Mellitus, Type 1/genetics , Diabetes Mellitus, Type 1/immunology , Immune Tolerance , Polyendocrinopathies, Autoimmune/immunology
14.
Rev. bras. anestesiol ; 65(2): 130-135, Mar-Apr/2015. tab, graf
Article in English | LILACS | ID: lil-741712

ABSTRACT

BACKGROUND AND OBJECTIVE: To investigate the influence of intraoperative and preoperative positive pressure in the time of extubation in patients undergoing bariatric surgery. METHOD: Randomized clinical trial, in which 40 individuals with a body mass index between 40 and 55 kg/m2, age between 25 and 55 years, nonsmokers, underwent bariatric surgery type Roux-en-Y gastric bypass by laparotomy and with normal preoperative pulmonary function were randomized into the following groups: G-pre (n = 10): individuals who received treatment with noninvasive positive pressure before surgery for 1 h; G-intra (n = 10): individuals who received positive end-expiratory pressure of 10 cm H2O throughout the surgical procedure; and G-control (n = 20): not received any preoperative or intraoperative intervention. Following were recorded: time between induction of anesthesia and extubation, between the end of anesthesia and extubation, duration of mechanical ventilation, and time between extubation and discharge from the post-anesthetic recovery. RESULTS: There was no statistical difference between groups. However, when applied to the Cohen coefficient, the use of positive end-expiratory pressure of 10 cm H2O during surgery showed a large effect on the time between the end of anesthesia and extubation. About this same time, the treatment performed preoperatively showed moderate effect. CONCLUSION: The use of positive end-expiratory pressure of 10 cm H2O in the intraoperative and positive pressure preoperatively, influenced the time of extubation of patients undergoing bariatric surgery. .


JUSTIFICATIVA E OBJETIVO: investigar a influência do uso da pressão positiva nas vias aéreas intraoperatória e pré-operatória no tempo de extubação de pacientes submetidos à cirurgia bariátrica. MÉTODO: Trata-se de ensaio clínico randomizado, no qual 40 indivíduos com índice de massa corporal entre 40 e 55 kg/m2, idade entre 25 e 55 anos, não tabagistas, submetidos à cirurgia bariátrica do tipo derivação gástrica em Y de Roux por laparotomia e com prova de função pulmonar pré-operatória dentro da normalidade foram randomizados nos seguintes grupos: G-pré (n = 10): indivíduos que receberam tratamento com pressão positiva não invasiva antes da cirurgia, durante uma hora, G-intra (n = 10): indivíduos que receberam Positive End-expiratory Pressure de 10 cm H2O durante todo o procedimento cirúrgico e G-controle (n = 20): não receberam qualquer tipo de intervenção pré ou intraoperatória. foram anotados os seguintes tempos: tempo decorrido entre a indução anestésica e a extubação, entre o término da anestesia e extubação, tempo de ventilação mecânica, e tempo entre a extubação e a alta da Recuperação Pós-Anestésica. RESULTADOS: Não houve diferença estatística entre os grupos, porém quando aplicado ao Coeficiente de Cohen, o uso da Positive End-expiratory Pressure de 10 cm H2O no intraoperatório mostrou um efeito grande sobre o tempo entre o término da anestesia e a extubação. Sobre este mesmo tempo, o tratamento realizado no pré-operatório apresentou efeito moderado. CONCLUSÃO: O uso da Positive End-expiratory Pressure de 10 cm H2O no intraoperatório e da pressão positiva no pré-operatório, pode influenciar o tempo de extubação de pacientes submetidos à cirurgia bariátrica. .


JUSTIFICACIÓN Y OBJETIVO: Investigar la influencia del uso de la presión positiva en las vías aéreas intraoperatoria y preoperatoria en el tiempo de extubación de pacientes sometidos a la cirugía bariátrica. MÉTODO: Se trata de un ensayo clínico aleatorizado, en el cual 40 individuos con IMC entre 40 y 55 kg/m2, edad entre 25 y 55 años, no fumadores, sometidos a cirugía bariátrica del tipo derivación gástrica en Y de Roux por laparotomía y con prueba de función pulmonar preoperatoria dentro de la normalidad fueron aleatorizados en los siguientes grupos: G-pre (n = 10): individuos que recibieron tratamiento con presión positiva no invasiva antes de la cirugía durante una hora; G-intra (n = 10): individuos que recibieron PEEP de 10 cm H2O durante todo el procedimiento quirúrgico y G-control (n = 20): no recibieron ningún tipo de intervención pre- o intraoperatoria. Fueron anotados los siguientes tiempos: tiempo trascurrido entre la inducción anestésica y la extubación, entre el fin de la anestesia y la extubación, tiempo de ventilación mecánica, y tiempo entre la extubación y el alta de la sala de recuperación postanestésica. RESULTADOS: No hubo diferencia estadística entre los grupos, sin embargo cuando se aplicó el coeficiente de Cohen, el uso de la PEEP de 10 cm H2O en el intraoperatorio mostró un efecto importante sobre el tiempo entre el término de la anestesia y la extubación. Sobre ese mismo tiempo, el tratamiento realizado en el preoperatorio presentó un efecto moderado. CONCLUSIÓN: El uso de la PEEP de 10 cm H2O en el intraoperatorio y de la presión positiva en el preoperatorio puede influir en el tiempo de extubación de pacientes sometidos a cirugía bariátrica. .


Subject(s)
Animals , Female , Humans , Male , Mice , Arthritis, Experimental/immunology , B-Lymphocyte Subsets/immunology , Wiskott-Aldrich Syndrome Protein/immunology , Arthritis, Experimental/genetics , Arthritis, Experimental/pathology , Autoimmune Diseases/genetics , Autoimmune Diseases/immunology , Autoimmune Diseases/pathology , B-Lymphocyte Subsets/pathology , /genetics , /immunology , Mice, Knockout , T-Lymphocytes, Regulatory/immunology , T-Lymphocytes, Regulatory/pathology , /immunology , /pathology , Wiskott-Aldrich Syndrome Protein/genetics
15.
Ciênc. saúde coletiva ; 20(1): 85-94, 01/2015. tab
Article in Portuguese | LILACS | ID: lil-733143

ABSTRACT

O presente estudo propõe-se a identificar a prevalência do acesso a informações sobre como evitar problemas bucais entre escolares da rede pública de ensino, assim como os fatores associados a este acesso. Trata-se de um estudo transversal e analítico conduzido entre escolares de 12 anos de idade de um município brasileiro de grande porte populacional. Os exames foram realizados por 24 cirurgiões-dentistas treinados e calibrados com auxilio de 24 anotadores. A coleta de dados ocorreu em 36 escolas sorteadas das 89 escolas públicas do município. Foram conduzidas análises descritivas, univariadas e múltiplas. Dos 2510 escolares incluídos no estudo, 2211 relataram já ter recebido informações sobre como evitar problemas bucais. O acesso a tais informações foi maior entre os que utilizaram serviços odontológicos privado/convênio; e menor entre aqueles que utilizaram o serviço para tratamento, os que avaliaram o serviço como regular ou ruim/péssimo, os que utilizam como meio de higiene bucal somente escova dente/escova dente e higienização a língua e os que relataram não estarem satisfeitos com a aparência de seus dentes. Conclui-se que a maioria dos escolares teve acesso a informações sobre como evitar problemas bucais, o qual esteve associado a características dos serviços de saúde, comportamentos e desfechos de saúde.


The scope of this study is to identify the prevalence of access to information about how to prevent oral problems among schoolchildren in the public school network, as well as the factors associated with such access. This is a cross-sectional and analytical study conducted among 12-year-old schoolchildren in a Brazilian municipality with a large population. The examinations were performed by 24 trained dentists and calibrated with the aid of 24 recorders. Data collection occurred in 36 public schools selected from the 89 public schools of the city. Descriptive, univariate and multiple analyses were conducted. Of the 2510 schoolchildren included in the study, 2211 reported having received information about how to prevent oral problems. Access to such information was greater among those who used private dental services; and lower among those who used the service for treatment, who evaluated the service as regular or bad/awful. The latter use toothbrush only or toothbrush and tongue scrubbing as a means of oral hygiene and who reported not being satisfied with the appearance of their teeth. The conclusion drawn is that the majority of schoolchildren had access to information about how to prevent oral problems, though access was associated with the characteristics of health services, health behavior and outcomes.


Subject(s)
Animals , Humans , Mice , Autoimmune Diseases/immunology , Autoimmunity/immunology , Mast Cells/immunology , Multiple Sclerosis/immunology , Central Nervous System/immunology , Dendritic Cells/immunology , Self Tolerance , T-Lymphocytes/immunology
16.
An. bras. dermatol ; 89(6): 885-889, Nov-Dec/2014. tab, graf
Article in English | LILACS | ID: lil-727652

ABSTRACT

BACKGROUND: Immunofluorescence testing is an important tool for diagnosing blistering diseases. OBJECTIVE: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases. METHODS: We retrospectively analyzed immunofluorescence results encompassing a 10-year period. RESULTS: 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate. CONCLUSION: Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect ...


Subject(s)
Female , Humans , Male , Autoimmune Diseases/diagnosis , Fluorescent Antibody Technique/methods , Skin Diseases, Vesiculobullous/diagnosis , Autoimmune Diseases/immunology , Immunoglobulin A/immunology , Immunoglobulin G/immunology , Reproducibility of Results , Retrospective Studies , Sensitivity and Specificity , Skin Tests , Skin Diseases, Vesiculobullous/immunology
19.
Rev. bras. reumatol ; 54(1): 13-20, Jan-Feb/2014. tab, graf
Article in Portuguese | LILACS | ID: lil-704281

ABSTRACT

Objetivo: Definir o título anormal e a diluição de triagem adequada para o teste de FAN (fator antinúcleo) por imunofluorescência indireta em células HEp-2 (FAN HEp-2). Métodos: Realizamos a pesquisa do FAN HEp-2 em amostras de soro de 126 indivíduos saudáveis. As amostras foram triadas na diluição de 1:80, e aquelas positivas diluídas até o título de 1:5120. O título anormal de FAN foi definido como aquele correspondente ao percentil 95 do teste nesta população. A sensibilidade dos diferentes títulos do FAN foi determinada em um grupo de 136 pacientes com diagnóstico de doença reumática autoimune, e a especificidade em um grupo de 118 pacientes com diagnóstico de outras doenças reumáticas. O valor de corte ótimo do teste foi determinado pelo estudo da curva ROC. Resultados: A frequência de FAN positivo em indivíduos saudáveis foi de 13,2%. Não houve diferença na frequência de resultados positivos de acordo com o gênero ou a idade. O título anormal do FAN foi definido como a diluição de 1:160. A diluição dos soros de 1:80 apresentou sensibilidade de 87,7% e especificidade de 67,8%, enquanto a diluição de 1:160 apresentou sensibilidade de 82% e especificidade de 73,7%. Pela análise da curva ROC, a diluição de 1:160 correspondeu ao valor de corte ótimo. Conclusão: O título anormal e o valor de corte ótimo do FAN HEp-2 na população avaliada foram de 1:160. A diluição de 1:160 é, portanto, a diluição de triagem ideal, com melhor especificidade, porém sem comprometimento significativo da sensibilidade diagnóstica do teste. .


Objective: To establish the abnormal title and the appropriate screening dilution for ANA (antinuclear antibodies) test by indirect immunofluorescence on HEp-2 cells (ANA HEp-2). Methods: An analysis of ANA Hep-2 in serum samples from 126 healthy individuals was performed. The samples were screened at a dilution of 1:80, and those positive were diluted to the title of 1:5120. The abnormal title of ANA was defined as that corresponding to the 95th percentile of the test in this population. The sensitivity of the different titles of antinuclear antibodies was determined in a group of 136 patients with a diagnosis of autoimmune rheumatic disease, and the specificity was determined in a group of 118 patients with other rheumatic diseases. The optimal cutoff value of the test was determined by ROC curve analysis. Results: The frequency of ANA positivity in healthy subjects was 13.2%. There was no difference in the frequency of positive results according to gender or age. The abnormal title of ANA was defined as the dilution of 1:160. The 1:80 dilution had sensitivity of 87.7% and specificity of 67.8%, while the 1:160 dilution had sensitivity of 82% and specificity of 73.7%. By ROC curve analysis, a dilution of 1:160 corresponded to the optimal cutoff value. Conclusion: The abnormal title and the optimal cutoff value of ANA HEp-2 in the population was 1:160. Therefore, the dilution of 1:160 is the optimal screening dilution, with better specificity but without significantly compromising the sensitivity of the diagnostic test. .


Subject(s)
Adult , Female , Humans , Male , Middle Aged , Antibodies, Antinuclear/blood , Antibodies, Antinuclear/isolation & purification , Autoimmune Diseases/blood , Autoimmune Diseases/diagnosis , Rheumatic Diseases/blood , Rheumatic Diseases/diagnosis , Autoimmune Diseases/immunology , Cell Line, Tumor , Epithelial Cells/classification , Epithelial Cells/immunology , Fluorescent Antibody Technique, Indirect , ROC Curve , Rheumatic Diseases/immunology
20.
Gut and Liver ; : 462-470, 2014.
Article in English | WPRIM | ID: wpr-108136

ABSTRACT

Recent studies have proposed nomenclatures of type 1 autoimmune pancreatitis (AIP) (IgG4-related pancreatitis), IgG4-related sclerosing cholangitis (IgG4-SC), IgG4-related cholecystitis, and IgG4-related hepatopathy as IgG4-related disease (IgG4-RD) in the hepato-bilio-pancreatic system. In IgG4-related hepatopathy, a novel concept of IgG4-related autoimmune hepatitis (AIH) with the same histopathological features as AIH has been proposed. Among organs involved in IgG4-RD, associations with pancreatic and biliary lesions are most frequently observed, supporting the novel concept of "biliary diseases with pancreatic counterparts." Targets of type 1 AIP and IgG4-SC may be periductal glands around the bile and pancreatic ducts. Based on genetic backgrounds, innate and acquired immunity, Th2-dominant immune status, regulatory T (Treg) or B cells, and complement activation via a classical pathway may be involved in the development of IgG4-RD. Although the role of IgG4 remains unclear in IgG4-RD, IgG4-production is upregulated by interleukin 10 from Treg cells and by B cell activating factor from monocytes/basophils with stimulation of toll-like receptors/nucleotide-binding oligomerization domain-like receptors. Based on these findings, we have proposed a hypothesis for the development of IgG4-RD in the hepato-bilio-pancreatic system. Further studies are necessary to clarify the pathogenic mechanism of IgG4-RD.


Subject(s)
Adaptive Immunity , Autoimmune Diseases/immunology , B-Cell Activating Factor/metabolism , Cholangitis, Sclerosing/immunology , Cholecystitis/immunology , Humans , Immunoglobulin G/immunology , Interleukin-10/metabolism , Liver Diseases/immunology , Pancreatitis/immunology , T-Lymphocytes, Regulatory/immunology
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