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Arq. ciências saúde UNIPAR ; 24(3): 133-138, set-dez. 2020.
Article in Portuguese | LILACS | ID: biblio-1129455


Quando um indivíduo é exposto a antígenos eritrocitários não próprios, ocorre uma resposta imunológica, que leva à produção de anticorpos irregulares voltados contra esses antígenos. Esse processo é conhecido como aloimunização eritrocitária e acontece em decorrência de transfusões de sangue ou gestações incompatíveis. Na medicina transfusional a pesquisa de anticorpos irregulares é fundamental, pois a falha na detecção de um aloanticorpo pode provocar reações transfusionais, aloimunizações, anemias hemolíticas autoimunes e doença hemolítica perinatal. Este estudo tem por objetivo analisar a frequência de anticorpos irregulares de pacientes atendidos no Hemocentro Regional de Francisco Beltrão, Paraná, no ano de 2017. Os dados foram coletados a partir da revisão de registros em arquivos do Laboratório de Imunohematologia do Hemonúcleo. Foram avaliados dados de 49 protocolos de pacientes que apresentaram dificuldades transfusionais no ano de 2017. Dentre os pesquisados, 37 pacientes (75,5%) apresentaram anticorpos irregulares. Dentre os anticorpos anti-eritrocitários observados neste estudo, evidenciou-se a presença de doze pacientes com anti-D (27,2%), seis pacientes com anti-K (13,6%), quatro pacientes com anti-C (9,0%) e em seis pacientes (13,6%) foi observada a presença de autoanticorpos. Este estudo indica que, nos pacientes transfundidos, os anticorpos mais frequentes foram os aloanticorpos Anti-D do Sistema Rh, provavelmente devido ao seu alto grau de imunogenicidade. A prevalência desses anticorpos é semelhante a vários estudos encontrados na literatura.

When an individual is exposed to not-self red blood cell antigens, an immune response occurs, which leads to the production of irregular antibodies directed against these antigens. This process is known as erythrocyte alloimmunization and occurs as a result of blood transfusions or incompatible pregnancies. In transfusion medicine, the search for irregular antibodies is essential, since failure to detect an alloantibody can cause transfusion reactions, alloimmunizations, autoimmune hemolytic anemias, and perinatal hemolytic disease. This study aims at analyzing the frequency of irregular antibodies of patients seen at the Regional Blood Center of Francisco Beltrão, Paraná, in 2017. The data were collected from the review of records in files of the Immunohematology Laboratory of Hemonúcleo. Data from 49 protocols of patients who had transfusion difficulties in 2017 were evaluated. Among those surveyed, 37 patients (75.5%) had irregular antibodies. Among the anti-erythrocyte antibodies observed in this study, the presence of twelve patients with anti-D (27.2%), six patients with anti-K (13.6%), four patients with anti-C (9.0 %), and in six patients (13.6%) with the presence of autoantibodies were observed. This study indicates that, in transfused patients, the most frequent antibodies were the Rh System Anti-D alloantibodies, probably due to their high degree of immunogenicity. The prevalence of these antibodies is similar to several studies found in the literature.

Humans , Male , Female , Child , Adolescent , Adult , Middle Aged , Aged , Aged, 80 and over , Young Adult , Autoantibodies/immunology , Isoantibodies/immunology , Autoantibodies/isolation & purification , Blood Transfusion , Retrospective Studies , Sex Distribution , Age Distribution , Erythrocytes/immunology , Transfusion Reaction/immunology , Isoantibodies/isolation & purification , Antibodies/isolation & purification , Antibodies/immunology
Gac. méd. Méx ; 156(1): 67-77, ene.-feb. 2020. tab, graf
Article in English, Spanish | LILACS | ID: biblio-1249872


Resumen La hemofilia adquirida (HA) es un trastorno hemostático autoinmune ocasionado por autoanticuerpos dirigidos contra el factor VIII: C. En 52 % de los casos, la causa se desconoce o no se asocia con otra entidad patológica; en el resto, existen factores concomitantes: lupus, artritis reumatoide, cáncer, embarazo y medicamentos. En México no existe registro ni conciencia de la enfermedad entre el personal de salud. Los grupos de mayor incidencia son las mujeres en edad reproductiva y los individuos mayores de 70 años. Se caracteriza por hemorragia grave, sobre todo posterior a traumatismos y parto o cesárea, y equimosis grandes en tronco y extremidades. La sospecha es simple, basta que concurran hemorragia súbita, grave y un TTPa prolongado que no se corrige con plasma. El tratamiento consiste en lograr la hemostasia y erradicar el anticuerpo; lo primero se logra con el factor VII activado recombinante o concentrado del complejo de protrombínico activado. La ciclofosfamida, prednisona o rituximab sirven para erradicar el anticuerpo. La mayoría de los casos no son diagnosticados y la mortalidad es alta. Ya que los médicos desconocen el problema, no se sospecha, no se diagnostica y no se trata. Este documento revisa los datos más recientes de la HA y abunda en el diagnóstico y tratamiento.

Abstract Acquired hemophilia (AH) is an autoimmune hemostatic disorder mediated by autoantibodies directed against factor VIII: C. In 52% of cases, the cause is unknown or is not associated with other pathological entities; in the rest, there are concomitant factors: lupus, rheumatoid arthritis, cancer, pregnancy, and medications. In Mexico, there is not a registry of AH, and awareness of the disease among health personnel is low. The groups with the highest incidence are women of childbearing age and individuals older than 70 years. It is characterized by severe bleeding, especially after trauma and normal childbirth or cesarean delivery, and large ecchymoses in the trunk and extremities. The suspicion is simple, it just takes for sudden, severe hemorrhage and a prolonged activated partial thromboplastin time that is not corrected with plasma to concur in an individual. Treatment involves achieving hemostasis and eradicating the antibody. The former is achieved with recombinant activated factor VII or activated prothrombin complex concentrate. Cyclophosphamide, prednisone or rituximab are used to eradicate the antibody. Most cases of AH are not diagnosed, which translates into a high mortality rate. Given that awareness about the disease among physicians is low, it is not suspected, neither diagnosed, and nor is it treated. This document reviews the most recent data on AH and expands on its diagnosis and treatment.

Humans , Male , Female , Pregnancy , Adult , Middle Aged , Aged , Young Adult , Autoantibodies/immunology , Factor VIII/immunology , Hemophilia A/immunology , Pregnancy Complications, Hematologic/etiology , Prognosis , Ecchymosis/etiology , Hemophilia A/complications , Hemophilia A/therapy , Hemophilia A/epidemiology , Hemorrhage/etiology , Immunosuppressive Agents/therapeutic use
Rev. méd. Chile ; 148(2): 160-167, feb. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1115772


Background: Certain associated and specific myositis antibodies are related to certain clinical phenotypes of dermatomyositis (DM), disease severity and the presence of cancer. Aim: To describe the clinical profile of Chilean patients with DM and their associated and specific myositis antibodies. Material and Methods: Review of medical records of 15 patients with DM aged 31 to 72 years. Their clinical characteristics, laboratory tests and complementary tests were reviewed. In serum samples from each patient the presence of 16 specific antibodies was analyzed by immunoblot technique (Myositis Profile Euroline Blot test kit). Results: Fourteen (93.3%) patients had skin manifestations, five (33.3%) had pulmonary involvement, two (13.3%) had an associated cancer and nine (60%) had specific antibodies associated with myositis. Conclusions: These patients with DM had a clinical profile similar to what has been described elsewhere. The profile of myositis specific antibodies was different from reports in other populations.

Humans , Male , Female , Middle Aged , Aged , Autoantibodies/blood , Dermatomyositis/diagnosis , Skin/immunology , Skin/pathology , Autoantibodies/immunology , Dermatomyositis/etiology , Dermatomyositis/blood
Arch. endocrinol. metab. (Online) ; 64(1): 66-70, Jan.-Feb. 2020. tab, graf
Article in English | LILACS | ID: biblio-1088765


ABSTRACT Objective Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder that is frequently seen in the eastern Mediterranean region. The thyroid gland can be affected in FMF patients through autoimmunity or amyloidosis. Here, we aimed to evaluate the structure and functions of the thyroid gland in addition to possible autoimmunity in FMF patients. Subjects and methods The study was conducted by the Endocrinology and Metabolism and Internal Medicine Departments. Thirty FMF patients and 30 age and gender-matched healthy controls were enrolled in the study. Free thyroxin (fT4), free triiodothyronine (fT3), thyroid-stimulating hormone (TSH), and anti-thyroid peroxidase (anti-TPO) autoantibodies were investigated. Detailed thyroid grayscale and Doppler Ultrasonography examinations and shear-wave elastosonography (SWE) were performed in the patient and control groups. Results Anti-TPO was detected in 24% (n = 7) of the patients. On the grayscale US, mean thyroid volumes were similar between the FMF and the control groups (p > 0.05). By Doppler US, thyroid vascularity observed was detected in 10.3% (n = 3) of the patients. SWE revealed that the mean velocity value of right vs. left lobe in the patient group was 1.77 ± 0.45 m/s and 1.95 ± 0.51 m/s, respectively. Compared to the control group, the mean velocity values were significantly higher in the right (p = 0.004) and left (p = 0.01) lobes of the patient group. The mean stiffness value in the patient group was also significantly higher in the right and left lobes [10.13 ± 5.65 kPa (p = 0.005) and 12.24 ± 6.17 kPa (p = 0.02), respectively]. Conclusion Recognizing the complications of FMF early in the course of the disease is as important as the early diagnosis of the disorder. Based on this, thyroid functions and changes in its structure should be evaluated carefully for early diagnosis of a possible coexisting thyroid disorder. Arch Endocrinol Metab. 2020;64(1):66-70

Humans , Male , Female , Adult , Familial Mediterranean Fever/physiopathology , Familial Mediterranean Fever/immunology , Autoantibodies/immunology , Autoimmunity/immunology , Familial Mediterranean Fever/diagnostic imaging , Autoantibodies/blood , Thyroid Gland/immunology , Triiodothyronine/immunology , Triiodothyronine/blood , Thyrotropin/immunology , Thyrotropin/blood , Case-Control Studies , Ultrasonography, Doppler , Iodide Peroxidase/immunology , Iodide Peroxidase/blood
Braz. j. med. biol. res ; 53(2): e8917, 2020. graf
Article in English | LILACS | ID: biblio-1055492


This study investigates the effect of the overexpression of the placental growth factor (PGF) and hyperoxia on lung development and determines whether anti-PGF antibody ameliorates hyperoxia-mediated impairment of lung development in newborn rats. After exposure to normoxic conditions for seven days, newborn rats subjected to normoxia were intraperitoneally or intratracheally injected with physiological saline, adenovirus-negative control (Ad-NC), or adenovirus-PGF (Ad-PGF) to create the Normoxia, Normoxia+Ad-NC, and Normoxia+Ad-PGF groups, respectively. Newborn rats subjected to hyperoxia were intraperitoneally injected with physiological saline or anti-PGF antibodies to create the Hyperoxia and Hyperoxia+anti-PGF groups, respectively. Our results revealed significant augmentation in the levels of PGF and its receptor Flt-1 in the lung tissues of newborn rats belonging to the Normoxia+Ad-PGF or Hyperoxia groups. PGF overexpression in these groups caused lung injury in newborn rats, while anti-PGF antibody treatment significantly cured the hyperoxia-induced lung injury. Moreover, PGF overexpression significantly increased TNF-α and Il-6 levels in the bronchoalveolar lavage (BAL) fluid of the Normoxia+Ad-PGF and Hyperoxia groups. However, their levels were significantly reduced in the BAL fluid of the Hyperoxia+anti-PGF group. Immunohistochemical analysis revealed that PGF overexpression and hyperoxia treatment significantly increased the expression of the angiogenesis marker, CD34. However, its expression was significantly decreased upon administration of anti-PGF antibodies (compared to the control group under hyperoxia). In conclusion, PGF overexpression impairs lung development in newborn rats while its inhibition using an anti-PGF antibody ameliorates the same. These results provided new insights for the clinical management of bronchopulmonary dysplasia in premature infants.

Animals , Female , Pregnancy , Rats , Autoantibodies/metabolism , Hyperoxia/metabolism , Lung Injury/metabolism , Placenta Growth Factor/metabolism , Antibodies, Monoclonal/metabolism , Autoantibodies/immunology , Microscopy, Electron, Scanning , Hyperoxia/complications , Hyperoxia/diagnostic imaging , Disease Models, Animal , Lung Injury/pathology , Lung Injury/diagnostic imaging , Placenta Growth Factor/immunology , Animals, Newborn , Antibodies, Monoclonal/immunology
Rev. chil. pediatr ; 90(5): 555-558, oct. 2019. graf
Article in Spanish | LILACS | ID: biblio-1058183


Resumen: En los últimos años se ha intentado comprender la etiología del Trastorno del Espectro Autista (TEA), evidenciandose que existe una compleja interacción entre factores genéticos y ambientales. Estudios epidemiológicos y en modelos animales sugieren que la activación inmune de la madre durante el embarazo puede asociarse un mayor riesgo de desarrollar TEA en los hijos, destacando el rol de las citoquinas proinflamatorias, los auto-anticuerpos y el rol de la microglia activada en la poda sináptica durante el desarrollo embrionario. Comprender mejor los factores asociados con los Trastornos del Neurodesarrollo permitirá en el futuro desarrollar estrategias de manejo y detección precoz en población de riesgo.

Abstract: Autism Spectrum Disorder (ASD) etiology has been related whit complex interaction between ge netic and environmental factors. In the last years, numerous studies have suggested that maternal immune activation during pregnancy could be related to ASD in the offspring. This relation could be explained by the effects of pro-inflammatory cytokines, autoantibodies and microglial synap tic pruning during early embryonic development. Better understanding of Neurodevelopmental Disorders risk factors will support appropriate strategies of screening and management of risk population.

Humans , Female , Pregnancy , Pregnancy Complications/immunology , Prenatal Exposure Delayed Effects/immunology , Autism Spectrum Disorder/immunology , Autoantibodies/immunology , Risk Factors , Cytokines/immunology , Microglia/immunology , Autism Spectrum Disorder/etiology
Medicina (B.Aires) ; 79(supl.3): 60-65, set. 2019. tab
Article in Spanish | LILACS | ID: biblio-1040552


La neuromielitis óptica (NMO) es un trastorno autoinmune, inflamatorio y desmielinizante del sistema nervioso central con predilección por los nervios ópticos y médula espinal. En el año 2004 se publicó la asociación de NMO con un anticuerpo contra el canal de agua acuaporina 4 (anti-AQP4), como una enfermedad diferente de la esclerosis múltiple (EM). Actualmente se propone el término trastornos del espectro NMO (NMOSD), debido a que las manifestaciones de la enfermedad pueden ser más extensas, afectando además del nervio óptico y médula espinal, al área postrema del bulbo raquídeo, tronco encefálico, diencéfalo y áreas cerebrales típicas (periependimarias, cuerpo calloso, cápsula interna y sustancia blanca subcortical). NMOSD se aplica también a pacientes que cumplen los criterios de NMO y son negativos para anti-AQP4. Dentro de este último grupo se ha detectado en un 20% la presencia de otro anticuerpo, anti-MOG (Glicoproteína oligodendrocítica de mielina) con un mecanismo fisiopatológico diferente pero con una clínica, en algunos casos, similar, y en general con mejor pronóstico. El tratamiento inmunosupresor en la crisis, así como el tratamiento a largo plazo en los casos que esté indicado, es fundamental para evitar secuelas y recidivas. El diagnóstico correcto de esta entidad es fundamental ya que puede ser agravado con el uso de fármacos útiles en el tratamiento de EM. En esta publicación haremos una revisión de la fisiopatología, clínica y criterios diagnósticos de NMOSD, y discutiremos las distintas opciones terapéuticas.

Neuromyelitis optica (NMO) is an autoimmune, inflammatory and de myelinat ing disorder of the central nervous system with a predilection for the optic nerves and spinal cord. In 2004 the association of NMO with an antibody against the water channel aquaporin 4 (anti-AQP4) was published as a different pathology from multiple sclerosis (MS). Currently the term NMO spectrum disorders (NMOSD) is proposed, because the manifestations of the disease can be more extensive, affecting in addition to the optic nerve and spinal cord, the area postrema of the dorsal medulla, brainstem, diencephalon and typical brain areas (periependymal, corpus callosum, internal capsule and subcortical white matter). NMOSD is also applied to patients who meet the NMO criteria and are negative for AQP4-IgG. Within the latter group, the presence of another antibody, anti-MOG, has been detected in 20%, with a different physiopathological mechanism, but with a similar clinic and a better prognosis. The immunosuppressive treatment in the attack, as well as the long-term treatment in the cases that are indicated, is fundamental to avoid sequelaes and recurrences. The correct diagnosis of this entity is essential since it can be aggravated with the use of drugs useful in the treatment of MS. In this publication we will review the pathophysiology, clinical and diagnostic criteria of NMOSD, and discuss the different therapeutic options.

Humans , Autoantibodies/immunology , Neuromyelitis Optica/diagnosis , Neuromyelitis Optica/drug therapy , Autoantibodies/adverse effects , Neuromyelitis Optica/physiopathology , Neuromyelitis Optica/immunology , Diagnosis, Differential , Multiple Sclerosis/diagnosis
An. bras. dermatol ; 94(4): 388-398, July-Aug. 2019. tab, graf
Article in English | LILACS | ID: biblio-1038294


Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.

Humans , Paraneoplastic Syndromes/pathology , Paraneoplastic Syndromes/therapy , Pemphigus/immunology , Pemphigus/pathology , Pemphigus/therapy , Paraneoplastic Syndromes/immunology , Skin/pathology , Autoantibodies/immunology , Pemphigus/diagnosis , Erythema/diagnosis , Erythema/pathology , Mouth Diseases/diagnosis , Mouth Diseases/pathology
An. bras. dermatol ; 94(3): 264-278, May-June 2019. tab, graf
Article in English | LILACS | ID: biblio-1011108


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

Humans , Male , Female , Adult , Middle Aged , Pemphigus/diagnosis , Skin/pathology , Autoantibodies/immunology , Surveys and Questionnaires , Pemphigus/classification , Pemphigus/therapy , Pemphigus/epidemiology , Immunoglobulins, Intravenous/therapeutic use , Desmosomes/immunology , Diagnosis, Differential , Immunosuppressive Agents/classification , Immunosuppressive Agents/therapeutic use , Immunotherapy/methods
Arch. endocrinol. metab. (Online) ; 62(6): 609-614, Dec. 2018. tab, graf
Article in English | LILACS | ID: biblio-983815


ABSTRACT Objective: The conversion of Hashimoto's thyroiditis (HT) to hyperthyroidism due to thyrotropin receptor antibodies is intriguing and considered rare. The contribution of TSH receptor blocking antibodies (TRAb), which may be stimulators (TSAb) or blockers (TBAb), is suspected. We describe clinical and biological variables in a series of patients switching from Hashimoto's thyroiditis to Grave's disease. Subjects and methods: Retrospective case study of 24 patients with Hashimoto's thyroiditis followed during 48 ± 36 months that developed later Graves' disease (GD). These variables were analysed in the hypo and hyperthyroid phase: age, sex, initial TSH, free triiodothyronine (fT3), free thyroxine (fT4), anti-TPO, TBII antibodies, parietal cell autoantibodies, time between hypo and hyperthyroidism, thyroid volume and levothyroxine doses (LT). Results: In HT, mean TSH was 9.4 ± 26.1 UI/L and levothyroxine treatment was 66.2 ± 30.8 µg/day. The switch to GD was observed 38 ± 45 months after HT diagnosis. As expected, we found significant differences on TSH, FT3, FT4 and TBAb levels. Three out of 14 patients had parietal cell autoantibodies. In two of these three cases there was an Helicobacter pylori infection. There were no significant differences between HT and GD groups with respect to thyroid volume. Conclusions: To our knowledge, large series documenting the conversion of HT to GD are scarce. Although rare, this phenomenon should not be misdiagnosed. Suspicion should be raised whenever thyroxine posology must be tapered down during the follow-up of HT patients. Further immunological and genetic studies are needed to explain this unusual autoimmune change.

Humans , Male , Female , Adult , Middle Aged , Young Adult , Receptors, Thyrotropin/immunology , Graves Disease/immunology , Hashimoto Disease/immunology , Autoantibodies/immunology , Thyroid Function Tests , Thyroxine/administration & dosage , Thyroxine/blood , Triiodothyronine/blood , Receptors, Thyrotropin/blood , Thyrotropin/blood , Graves Disease/blood , Retrospective Studies , Statistics, Nonparametric , Immunoglobulins, Thyroid-Stimulating/immunology , Hashimoto Disease/blood , Hypothyroidism/immunology , Luminescent Measurements
Medicina (B.Aires) ; 78(5): 360-363, oct. 2018. ilus
Article in Spanish | LILACS | ID: biblio-976125


La dematomiositis es una miopatía inflamatoria idiopática con espectro clínico variable. En los últimos años se ha identificado un número de autoanticuerpos específicos de miositis útiles para el diagnóstico, la clasificación y el pronóstico de las diversas formas de la enfermedad, entre los que se encuentra el anti-MDA5. Este anticuerpo se asocia al desarrollo de úlceras cutáneas, enfermedad intersticial pulmonar rápidamente progresiva, mortalidad temprana y mal pronóstico por lo que la detección del mismo, en un contexto clínico adecuado, plantea la necesidad de un tratamiento inmunosupresor agresivo. Describimos un caso de dermatomiositis hipomiopática, (es decir, con afección muscular leve) que presentaba compromiso cutáneo específico, enfermedad pulmonar intersticial y anticuerpo anti-MDA5 que respondió favorablemente al tratamiento combinado con ciclofosfamida, gamaglobulina y corticoides.

Dematomyositis is an idiopathic inflammatory myopathy with a variable clinical spectrum. In recent years, a number of myositis-specific antibodies have been identified including anti-MDA5, which is us eful for diagnosis, prognosis and classification of the diverse clinical forms of the disease. This antibody is associated with cutaneous ulcers, rapidly progressive interstitial lung disease, early mortality and poor prognosis, so the detection of this antibody in a suitable clinical context, raises the need for an aggressive immunosuppressive treatment. We describe a case of dermatomyositis classified as hypomyopathic (i.e. involving mild muscle weakness), presenting specific skin lesions, interstitial lung disease, and presence of anti-MDA5 antibody that had a favorable response to combined treatment with cyclophosphamide, gamma globulin and corticosteroids.

Humans , Female , Adult , Autoantibodies/immunology , Lung Diseases, Interstitial/immunology , Dermatomyositis/immunology , Interferon-Induced Helicase, IFIH1/immunology , Prognosis , Biopsy , Tomography, X-Ray Computed , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/pathology , Dermatomyositis/diagnosis , Dermatomyositis/pathology
An. bras. dermatol ; 93(5): 638-650, Sept.-Oct. 2018. tab, graf
Article in English | LILACS | ID: biblio-949951


Abstract: Fogo selvagem or endemic pemphigus foliaceus is an autoimmune acantholytic anti-cadherin bullous disease that primarily affects seborrheic areas, which might disseminate. Brazil has the world's largest number of patients, mainly in the Central-West region, but the disease has also been reported in other South American countries. It affects young people and adults who have been exposed to rural areas, with occurrence of familial cases. Anti-desmoglein-1 autoantibodies are directed against desmosomal structures, with loss of adhesion of the upper layers of the epidermis, causing superficial blisters. The etiology is multifactorial and includes genetic, immune, and environmental factors, highlighting hematophagous insect bites; drug-related factors are occasionally involved. Flaccid blisters readily rupture to yield erosive-crusty lesions that sometimes resemble seborrheic dermatitis, actinic keratosis, and chronic cutaneous lupus erythematosus. The clinical presentation varies from localized to disseminated lesions. Clinical suspicion should be confirmed with histopathological and immunofluorescence tests, among others. The progression is usually chronic, and therapy varies according to clinical presentation, but generally requires systemic corticosteroid therapy associated with adjuvant immunosuppressive treatment to decrease the adverse effects of corticosteroids. Once the disease is under control, many patients remain stable on low-dose medication, and a significant proportion achieve remission.

Humans , Pemphigus/etiology , Pemphigus/epidemiology , Endemic Diseases , Autoantibodies/immunology , Brazil/epidemiology , Photography , Pemphigus/diagnosis , Pemphigus/pathology , Desmogleins/immunology
An. bras. dermatol ; 93(4): 517-523, July-Aug. 2018. tab
Article in English | LILACS | ID: biblio-949930


Abstract: Background: There have been no studies to date on the frequency and reactivity of aanti-melanoma differentiation-associated gene 5 (anti-MDA-5) in samples from the Brazilian population with dermatomyositis. Objectives: To analyze this autoantibody in the Brazilian population. Methods: This was a single-center cross-sectional study in which 131 consecutive adult patients (109 dermatomyositis and 22 clinically amyopathic dermatomyositis) with active disease were evaluated from 2000 to 2016. Analysis of the anti-MDA-5 autoantibody was performed by ELISA. Results: The presence of this autoantibody was observed in 14.7% and 22.7% of patients with dermatomyositis and clinically amyopathic dermatomyositis, respectively. In the case of dermatomyositis, the autoantibody was associated less frequently with Raynaud's phenomenon and periungual hyperemia (P<0.05). In clinically amyopathic dermatomyositis, the presence of this autoantibody was not associated statistically with any demographic, clinical, laboratory, or imaging characteristics. Study limitations: The cross-sectional study design did not allow establishing a temporal correlation between anti-MDA-5 autoantibody and various study variables. In addition, pulmonary function tests were not performed in the patients. Conclusions: The frequency of anti-MDA-5 autoantibody was comparable to that of other populations with dermatomyositis, but with a different reactivity than described in the literature. In addition, there was a phenotypic variability between our patients with clinically amyopathic dermatomyositis and those described in the literature. Further studies are needed to confirm the current study's findings and elucidate this autoantibody's reactivity in Brazilians with idiopathic inflammatory myopathies.

Humans , Male , Female , Adult , Autoantibodies/blood , Dermatomyositis/immunology , Interferon-Induced Helicase, IFIH1/immunology , Autoantibodies/immunology , Severity of Illness Index , Enzyme-Linked Immunosorbent Assay , Cross-Sectional Studies , Statistics, Nonparametric , Disease Progression , Dermatomyositis/complications , Dermatomyositis/blood , Interferon-Induced Helicase, IFIH1/blood
Arch. endocrinol. metab. (Online) ; 62(1): 34-40, Jan.-Feb. 2018. tab, graf
Article in English | LILACS | ID: biblio-887623


ABSTRACT Objective The aim of this research was to analyze the expression profile of miR-155, miR-146a, and miR-326 in peripheral blood mononuclear cells (PBMC) of 47 patients with type 1 diabetes mellitus (T1D) and 39 control subjects, as well as the possible association with autoimmune or inflammatory markers. Subjects and methods Expression profile of miRs by means of qPCR using TaqMan probes. Autoantibodies and inflammatory markers by ELISA. Statistical analysis using bivariate correlation. Results The analysis of the results shows an increase in the expression of miR-155 in T1D patients in basal conditions compared to the controls (p < 0.001) and a decreased expression level of miR-326 (p < 0.01) and miR-146a (p < 0.05) compared T1D patients to the controls. miR-155 was the only miRs associated with autoinmmunity (ZnT8) and inflammatory status (vCAM). Conclusion Our data show a possible role of miR-155 related to autoimmunity and inflammation in Chilean patients with T1D.

Humans , Child , MicroRNAs/metabolism , Diabetes Mellitus, Type 1/metabolism , Autoantibodies/immunology , Autoantibodies/metabolism , Enzyme-Linked Immunosorbent Assay , Biomarkers , Autoimmunity/immunology , Case-Control Studies , Diabetes Mellitus, Type 1/immunology , Diabetes Mellitus, Type 1/blood , Real-Time Polymerase Chain Reaction , Inflammation/immunology , Inflammation/metabolism
Medwave ; 18(6): e7298, 2018.
Article in English, Spanish | LILACS | ID: biblio-948400


Resumen La encefalopatía de Hashimoto es una enfermedad rara. Se reporta una prevalencia de 2,1 por cada 100 000 habitantes. Entre las manifestaciones clínicas se describen confusión, disminución del estado de consciencia, déficit cognitivo, convulsiones, mioclonus, ataxia y/o déficits neurológicos focales. Debido a la amplia variedad de signos y síntomas, la sospecha clínica diagnóstica es fundamental. El diagnóstico se basa en tres pilares: la presencia de manifestaciones clínicas neurológicas, con la exclusión de otras causas de encefalopatía; presencia de anticuerpos antitiroideos aumentados; una mejoría clínica notable luego de la administración de inmunomoduladores. El tratamiento de la encefalopatía de Hashimoto tiene dos objetivos: controlar el proceso autoinmune y controlar las complicaciones de la enfermedad. Aunque en la mayoría de los casos la recuperación es completa con el tratamiento, el riesgo de recaídas puede oscilar entre 12,5 a 40% en seguimientos a dos años.

Abstract Hashimoto's encephalopathy is a rare disease, with a reported prevalence of 2.1 per 100 000. Clinical manifestations include confusion, decreased state of consciousness, cognitive deficit, seizures, myoclonus, ataxia, and focal neurological deficits. Due to the wide variety of signs and symptoms, clinical diagnostic suspicion is essential. Diagnosis is based on three pillars: the presence of neurological clinical manifestations after ruling out other causes of encephalopathy. 2) Presence of increased antithyroid antibodies. 3) Significant clinical improvement after the administration of immunomodulation. The treatment of Hashimoto's encephalopathy pursues two objectives: to control the autoimmune process and to control the complications of the disease. Although in most cases recovery is complete with treatment, the risk of relapse can range from 12.5 to 40% in follow-ups to 2 years.

Humans , Autoantibodies/immunology , Encephalitis/therapy , Hashimoto Disease/therapy , Immunologic Factors/therapeutic use , Recurrence , Treatment Outcome , Encephalitis/diagnosis , Encephalitis/physiopathology , Hashimoto Disease/diagnosis , Hashimoto Disease/physiopathology
Säo Paulo med. j ; 135(6): 535-540, Nov.-Dec. 2017. tab
Article in English | LILACS | ID: biblio-904120


ABSTRACT BACKGROUND: Thyroid autoimmunity is more common in patients with rheumatic diseases than in healthy populations. The degree of association seems subject to influence from patients' geographical location. Here, we aimed to ascertain the prevalence of thyroid autoantibodies in a cohort of patients with systemic rheumatic disease and the degree of association between its presence and inflammatory activity. DESIGN AND SETTING: Cross-sectional observational study in a rheumatology unit. METHODS: 301 patients with systemic lupus erythematosus (SLE), 210 with rheumatoid arthritis (RA), 58 with scleroderma (SSc) and 80 with spondyloarthritis (SpA) were studied regarding thyroid function (TSH and T4), anti-thyroglobulin (TgAb) and anti-thyroperoxidase (TPOab) and compared with 141 healthy controls. Disease activity in patients with rheumatic disease was assessed through appropriate indexes. RESULTS: There were more antithyroid antibodies in SLE patients with hypothyroidism (P = 0.01; odds ratio, OR 2.7; 95% confidence interval, CI: 1.20-6.26) and in those without hypothyroidism (P = 0.06; OR 2.4; 95% CI: 1.28-4.55) than in controls. SSc patients also showed: P = 0.03 both with antithyroid antibodies and hypothyroidism (OR 3.4; 95% CI: 1.06-10.80) and without hypothyroidism (OR 3.1; 95% CI: 1.11-0.13). RA and SpA patients had the same prevalence as controls (P not significant). Presence of autoantibodies with and without hypothyroidism was not associated with the activity or functional indexes evaluated. CONCLUSION: SLE and SSc were associated with higher prevalence of thyroid autoantibodies in patients with and without hypothyroidism, unlike SpA and RA. There was no link between thyroid autoantibody presence and disease activity or functional impairment.

Humans , Male , Female , Adult , Middle Aged , Autoantibodies/blood , Autoimmune Diseases/blood , Rheumatic Diseases/blood , Iodide Peroxidase/blood , Arthritis, Rheumatoid/immunology , Arthritis, Rheumatoid/blood , Scleroderma, Systemic/immunology , Scleroderma, Systemic/blood , Autoantibodies/immunology , Case-Control Studies , Rheumatic Diseases/immunology , Prevalence , Cross-Sectional Studies , Spondylarthropathies/immunology , Spondylarthropathies/blood , Disability Evaluation , Iodide Peroxidase/immunology , Lupus Erythematosus, Systemic/immunology , Lupus Erythematosus, Systemic/blood
Arq. neuropsiquiatr ; 75(10): 687-691, Oct. 2017. tab, graf
Article in English | LILACS | ID: biblio-888260


ABSTRACT Autoantibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been reported in patients with inflammatory central nervous system disorders including isolated optic neuritis (ON). We compared our MOG-IgG ON patients with multiple sclerosis (MS) patients presenting with ON. Methods and results: Among the total of 38 patients with optic neuropathies, six patients with isolated ON were MOG-IgG positive and eight patients with ON fulfilled the diagnostic criteria for MS. All MS patients were negative for MOG-IgG using a cell-based assay. When compared with the MS group, the MOG-IgG patients were older (mean 47 years), more frequently male (ratio 2:1) and had a higher frequency of bilateral and/or recurrent ON. The brain magnetic resonance imaging of all MOG-IgG positive patients was normal or had only unspecific white matter T2 lesions. Conclusion: These findings suggest that MOG-IgG is a biomarker of an inflammatory demyelinating CNS disease distinct from MS.

RESUMO Autoanticorpos contra a glicoproteína da mielina do oligodendrócito (MOG-IgG) têm sido descritos em pacientes com neurite óptica (NO) isolada, entre outras doenças inflamatórias do sistema nervoso central. Comparamos os nossos pacientes com NO MOG-IgG positivos com pacientes com NO associada a esclerose múltipla (EM). Materias e métodos: De um total de 38 pacientes com neuropatia óptica, seis foram MOG-IgG positivos e oito preencheram critérios diagnósticos para EM. Todos os pacientes com EM foram negativos para MOG-IgG (ensaio baseado em células). Quando comparados ao grupo com EM, os pacientes MOG-IgG positivos apresentam idade mais avançada (mediana de 47 anos) e tiveram uma frequência maior de NO bilateral e/ou recorrente. Houve predomínio masculino (relação 2:1). A ressonância magnética de encéfalo de todos os pacientes MOG-IgG positivos foi normal ou demonstrou apenas lesões inespecíficas em T2. Conclusão: Nossos achados sugerem que o MOG-IgG é um biomarcador de doença desmielinizante diferente da EM.

Humans , Male , Female , Adult , Middle Aged , Young Adult , Autoantibodies/blood , Biomarkers/blood , Optic Neuritis/blood , Myelin-Oligodendrocyte Glycoprotein/immunology , Multiple Sclerosis/blood , Autoantibodies/immunology , Magnetic Resonance Imaging , Optic Neuritis/complications , Myelin-Oligodendrocyte Glycoprotein/blood , Multiple Sclerosis/complications
Rev. bras. reumatol ; 57(1): 15-22, Jan.-Feb. 2017. tab
Article in English | LILACS | ID: biblio-844209


ABSTRACT Introduction: The presence of anti-CCP is an important prognostic tool of rheumatoid arthritis (RA). But research is still ongoing on its relationship with disease activity and functional capacity. Objectives: To study the relationship between anti-CCP and disease activity, functional capacity and structural damage indexes, by means of conventional radiography (CR) and magnetic resonance imaging (MRI), in cases of established RA. Methods: Cross-sectional study with RA patients with 1–10 disease duration. Participants underwent clinical evaluation with anti-CCP. Disease activity was assessed using the Clinical Disease Activity Index (CDAI), and functional capacity through the Health Assessment Questionnaire (HAQ). CR analysis was carried out by the Sharp van der Heijde index (SvdH), and MRI analysis by RAMRIS (Rheumatoid Arthritis Magnetic Resonance Image Scoring). Results: We evaluated 56 patients, with a median (IqR) age of 55 (47.5–60) years; 50 (89.3%) participants were female and 37 (66.1%) were positive for anti-CCP. Medians (IqR) of CDAI, HAQ, SvdH and RAMRIS were 14.75 (5.42–24.97) 1.06 (0.28–1.75), 2 (0–8) and 15 (7–35), respectively. There was no association between anti-CCP and CDAI, HAQ and SvdH and RAMRIS scores. Conclusion: Our results have not established an association of anti-CCP with the severity of disease. To date, we cannot corroborate anti-CCP as a prognostic tool in patients with established RA.

RESUMO Introdução: A presença do anti-CCP constitui importante ferramenta prognóstica da artrite reumatoide (AR), mas ainda se investiga sua relação com a atividade da doença e a a capacidade funcional. Objetivos: Estudar a relação do anti-CCP com os índices de atividade da doença, de capacidade funcional e de dano estrutural, por meio de radiografia convencional (RC) e de ressonância magnética (RM), em AR estabelecida. Métodos: Estudo transversal com pacientes com AR, com um a 10 anos de doença. Os participantes foram submetidos à avaliação clínica com pesquisa do anti-CCP. A atividade de doença foi avaliada por meio do Clinical Disease Activity Index (CDAI) e a capacidade funcional por meio do Health Assessment Questionnaire (HAQ). A análise da RC foi feita pelo índice de Sharp van der Heijde (SmvH) e da RM pelo Sistema de Pontuação de Imagem por Ressonância Magnética na Artrite Reumatoide (RAMRIS, Rheumatoid Arthritis Magnetic Resonance Image Scoring). Resultados: Foram avaliados 56 pacientes, com mediana (IIq) de 55 (47,5-60,0) anos, 50 (89,3%) do sexo feminino e 37 (66,1%) anti-CCP positivos. As medianas (IIq) do CDAI, do HAQ, de SmvH e do RAMRIS foram de 14,75 (5,42-24,97), 1,06 (0,28-1,75), 2 (0-8) e 15 (7-35), respectivamente. Não houve associação do anti-CCP com o CDAI, com o HAQ e com os escores SmvH e RAMRIS. Conclusão: Nossos resultados não estabeleceram a associação do anti-CCP com a gravidade da doença. Até o momento, não podemos corroborar o anti-CCP como uma ferramenta prognóstica em AR estabelecida.

Humans , Male , Peptides, Cyclic/immunology , Arthritis, Rheumatoid/immunology , Arthritis, Rheumatoid/diagnostic imaging , Autoantibodies/immunology , Magnetic Resonance Imaging , Peptides, Cyclic/blood , Arthritis, Rheumatoid/physiopathology , Arthritis, Rheumatoid/blood , Prognosis , Rheumatoid Factor/blood , Autoantibodies/blood , Severity of Illness Index , Biomarkers/blood , Cross-Sectional Studies , Predictive Value of Tests , Middle Aged
Rev. cuba. med ; 55(4): 324-339, oct.-dic. 2016. ilus
Article in Spanish | LILACS | ID: biblio-845003


La aceptación y la relevancia de los autoanticuerpos se evidencia por su creciente incorporación en los criterios diagnósticos y de clasificación de las enfermedades autoinmunes. Una de las preocupaciones actuales, derivadas del uso ampliado de los autoanticuerpos, es la conservación del uso adecuado, en contraposición con el uso indiscriminado que genera un aumento de resultados falsos positivos que conducen a costosos errores en el diagnóstico, seguimiento, e incluso, tratamiento del paciente. Este artículo intenta resumir las circunstancias en que es oportuno ordenar las pruebas de autoanticuerpos, y cómo interpretarlas para preservar su utilidad clínica y refrenar los gastos de salud(AU)

The acceptance and relevance of auto-antibodies is evidenced by their increasing incorporation into the diagnostic and classification criteria of autoimmune diseases. One of the current concerns arising from the widespread use of auto-antibodies is the observance of adequate use, as opposed to its undiscriminating use that results in an increase of false positive results leading to costly errors in diagnosis, follow-up, and even treatment of the patient. This article attempts to summarize the circumstances when it is timely to order autoantibody tests, and how to interpret them to preserve their clinical utility and control health expenditures(AU)

Humans , Male , Female , Autoantibodies/immunology , Autoimmune Diseases/diagnosis , Antibodies, Antinuclear/immunology , Immunologic Tests/methods