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INTRODUCTION: The epidemiology of Juvenile Dermatomyositis (JDM) in non-Caucasian population is poorly described. We performed a study of patients followed up in the French West Indies for JDM. We aimed to describe clinical and biological specificities during childhood. METHODS: Retrospective study covering the period from Januarys 2000-2023. Listings of patients were obtained from multiple sources, namely computerized hospital archives, registry of referent pediatricians and adult specialists in internal medicine and the French National Registry for rare diseases. JDM and organ involvement were defined according to the international ILAR criteria. RESULTS: Twenty-one patients were included over a 23 year-period. Median age at onset was 8.1 years (Range: 2.5-13.9) with a median follow up of 8 years (Range: 2-19). Two-thirds (14/21) had dysphagia at onset and 33% had respiratory involvement. Thirteen had specific autoantibodies (58%), most frequently anti-Mi-2. The median number of flares during childhood was three (1-9). During childhood, 76% had calcinosis lesions. Clinical evolution seemed to be more aggressive for boys than girls (respectively 4.2 versus 2.2 flares (p = 0.04) and 50% vs 18% needing more than one background therapy, p = 0.03). CONCLUSION: This retrospective study is the largest cohort of pediatric patients of Afro-Caribbean and Black African descent treated for JDM in a high-income health system, and the first to describe the incidence and immunological profile in a population of African descent. They had higher rate of calcinosis and similar respiratory involvement. Overall outcomes during childhood were similar to North America and European countries.
Subject(s)
Calcinosis , Dermatomyositis , Male , Adult , Female , Child , Humans , Child, Preschool , Adolescent , Cohort Studies , Retrospective Studies , West Indies/epidemiologyABSTRACT
Mesenchymal stromal cell (MSC)-derived products, such as trophic factors (MTFs), have anti-inflammatory properties that make them attractive for cell-free treatment. Three-dimensional (3D) culture can enhance these properties, and large-scale expansion using a bioreactor can reduce manufacturing costs. Three lots of MTFs were obtained from umbilical cord MSCs produced by either monolayer culture (Monol MTF) or using a 3D microcarrier in a spinner flask dynamic system (Bioreactor MTF). The resulting MTFs were tested and compared using anti-inflammatory potency assays in two different systems: (1) a phytohemagglutinin-activated peripheral blood mononuclear cell (PBMNC) system and (2) a lipopolysaccharide (LPS)-activated macrophage system. Cytokine expression by macrophages was measured via RT-PCR. The production costs of hypothetical units of anti-inflammatory effects were calculated using the percentage of TNF-α inhibition by MTF exposure. Bioreactor MTFs had a higher inhibitory effect on TNF (p < 0.01) than monolayer MTFs (p < 0.05). The anti-inflammatory effect of Bioreactor MTFs on IL-1ß, TNF-α, IL-8, IL-6, and MIP-1 was significantly higher than that of monolayer MTFs. The production cost of 1% inhibition of TNF-α was 11-40% higher using monolayer culture compared to bioreactor-derived MTFs. A 3D dynamic culture was, therefore, able to produce high-quality MTFs, with robust anti-inflammatory properties, more efficiently than monolayer static systems.
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Abstract Objectives Adherence problems have a great impact on auto-immune Rheumatic Diseases (AIRD). The COVID-19 pandemic may have worsened treatment adherence. The aims of this study were to measure treatment adherence to identify an earlier risk of poor adherence and measure families' satisfaction with the health service during the pandemic. Methods Prospective observational study with 50 parents/children and adolescents with recent AIRD diagnosis. Initially, they answered questions (demographic data, disease) and completed the Pediatric Rheumatology Adherence Questionnaire (PRAQ), after 6 months they completed the Morisky-Green Test (MGT), Brief Medication Questionnaire (BMQ), Compliance Questionnaire for Rheumatology (CQR-19) and Pediatric Quality of Life Inventory Questionnaire 3.0 (PedsQlTM-SSS). The patient's medical records from the previous 12 weeks were reviewed for global and medication adherence data. Results The mean global adherence score was 94.3 ± 10.0, for medication adherence 97.3 ± 9.3, and for PRAQ questionnaire 5.2 ± 1.5. The authors observed agreement between MGT, BMQ, CQR-19, PedsQLTM-SSS scores and medication adherence rate, but not with global adherence rate. There were no associations between demographic characteristics, disease diagnosis, and adherence. No associations between PRAQ scores and values and global/medication adherence rates were observed. No variables were shown to be predictors of good adherence. The mean PedsQLTM-SSS rate was 92.1 ± 6.8. Conclusion The high values of MGT, BMQ, CQR-19 questionnaire scores were in agreement with the medication adherence rate. Despite the pandemic, the global and medication adherence rates were good. It was not possible to demonstrate the PRAQ's predictive power. The authors weren't able to establish an association between families' satisfaction and treatment adherence rates.
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BACKGROUND: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune multi-system disorder frequently affecting black women of childbearing age. No published data exist on the obstetric outcomes in a Caribbean population. OBJECTIVE: We analyzed pregnancy outcomes in an Afro-Caribbean cohort of women with SLE at a tertiary university hospital. METHODS: A retrospective cohort study was performed of all pregnant women with SLE prior to pregnancy from January 1990 to December 2021 at the University Hospital of the West Indies (UHWI), Jamaica. Maternal rheumatologic, obstetric, fetal/neonatal data were analyzed. Descriptive statistical analyses were performed. To determine if outcomes were associated with various factors, Spearman's rho was followed by logistic regression analysis to estimate unadjusted odds ratios with statistical significance at p < 0.05. RESULTS: A total of 56 pregnancies in 47 women were identified with SLE. Live births were 87.5%, with 10.7% spontaneous miscarriages and no neonatal deaths. Prednisone was the most used drug in 67.9% of patients. 85% of women had an adverse outcome with an adverse fetal outcome occurring in 55% of cases. Prednisone was associated with an adverse fetal/neonatal outcome (Spearman's rho = 0.38; p = .004). CONCLUSION: In this first Caribbean series on SLE in pregnancy, reasonably successful pregnancy outcomes are achievable in Afro-Caribbean women managed in multidisciplinary centers.
Subject(s)
Lupus Erythematosus, Systemic , Pregnancy Complications , Female , Pregnancy , Humans , Pregnancy Outcome/epidemiology , Retrospective Studies , Prednisone , Pregnancy Complications/epidemiology , Lupus Erythematosus, Systemic/complications , West Indies , Caribbean RegionABSTRACT
OBJECTIVES: Adherence problems have a great impact on auto-immune Rheumatic Diseases (AIRD). The COVID-19 pandemic may have worsened treatment adherence. The aims of this study were to measure treatment adherence to identify an earlier risk of poor adherence and measure families' satisfaction with the health service during the pandemic. METHODS: Prospective observational study with 50 parents/children and adolescents with recent AIRD diagnosis. Initially, they answered questions (demographic data, disease) and completed the Pediatric Rheumatology Adherence Questionnaire (PRAQ), after 6 months they completed the Morisky-Green Test (MGT), Brief Medication Questionnaire (BMQ), Compliance Questionnaire for Rheumatology (CQR-19) and Pediatric Quality of Life Inventory Questionnaire 3.0 (PedsQlTM-SSS). The patient's medical records from the previous 12 weeks were reviewed for global and medication adherence data. RESULTS: The mean global adherence score was 94.3 ± 10.0, for medication adherence 97.3 ± 9.3, and for PRAQ questionnaire 5.2 ± 1.5. The authors observed agreement between MGT, BMQ, CQR-19, PedsQLTM-SSS scores and medication adherence rate, but not with global adherence rate. There were no associations between demographic characteristics, disease diagnosis, and adherence. No associations between PRAQ scores and values and global/medication adherence rates were observed. No variables were shown to be predictors of good adherence. The mean PedsQLTM-SSS rate was 92.1 ± 6.8. CONCLUSION: The high values of MGT, BMQ, CQR-19 questionnaire scores were in agreement with the medication adherence rate. Despite the pandemic, the global and medication adherence rates were good. It was not possible to demonstrate the PRAQ's predictive power. The authors weren't able to establish an association between families' satisfaction and treatment adherence rates.
Subject(s)
COVID-19 , Rheumatic Diseases , Humans , Child , Adolescent , Pandemics , Quality of Life , COVID-19/epidemiology , Medication Adherence , Surveys and Questionnaires , Rheumatic Diseases/diagnosis , Rheumatic Diseases/drug therapyABSTRACT
Curcumin (CUR) is a polyphenol extracted from the rhizome of Curcuma longa that possesses potent anti-inflammatory and antioxidant potential. Despite CUR's numerous beneficial effects on human health, it has limitations, such as poor absorption. Nano-based drug delivery systems have recently been applied to improve CUR's solubility and bioavailability and potentialize its health effects. This review investigated the effects of different CUR-based nanomedicines on inflammatory and immunomodulated diseases. PUBMED, EMBASE, COCHRANE, and GOOGLE SCHOLAR databases were searched, and the Scale for Assessment of Narrative Review Articles (SANRA) was used for quality assessment and PRISMA guidelines. Overall, 66 studies were included comprising atherosclerosis, rheumatoid arthritis (RA), Alzheimer's disease (AD), Parkinson's disease (PD), multiple sclerosis (MS), Huntington's disease (HD), inflammatory bowel diseases (IBD), psoriasis, liver fibrosis, epilepsy, and COVID-19. The available scientific studies show that there are many known nanoformulations with curcumin. They can be found in nanosuspensions, nanoparticles, nanoemulsions, solid lipid particles, nanocapsules, nanospheres, and liposomes. These formulations can improve CUR bioavailability and can effectively be used as adjuvants in several inflammatory and immune-mediated diseases such as atheroma plaque formation, RA, dementia, AD, PD, MS, IBD, psoriasis, epilepsy, COVID-19, and can be used as potent anti-fibrotic adjuvants in fibrotic liver disease.
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This comprehensive review focuses on the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its impact as the cause of the COVID-19 pandemic. Its objective is to provide a cohesive overview of the epidemic history and evolutionary aspects of the virus, with a particular emphasis on its emergence, global spread, and implications for public health. The review delves into the timelines and key milestones of SARS-CoV-2's epidemiological progression, shedding light on the challenges encountered during early containment efforts and subsequent waves of transmission. Understanding the evolutionary dynamics of the virus is crucial in monitoring its potential for adaptation and future outbreaks. Genetic characterization of SARS-CoV-2 is discussed, with a focus on the emergence of new variants and their implications for transmissibility, severity, and immune evasion. The review highlights the important role of genomic surveillance in tracking viral mutations linked to establishing public health interventions. By analyzing the origins, global spread, and genetic evolution of SARS-CoV-2, valuable insights can be gained for the development of effective control measures, improvement of pandemic preparedness, and addressing future emerging infectious diseases of international concern.
ABSTRACT
Membranous nephropathy (MN) is a form of kidney disease that is idiopathic in 70%-80% of cases. Glomerular involvement in autoimmune thyroiditis can occur in 10%-30% of patients, and MN manifests in association with Hashimoto thyroiditis in up to 20% of the cases with glomerular involvement. Reports of MN associated with Graves' disease (GD) are extremely rare in the current literature. Herein, we report the case of a 46-year-old man admitted to the hospital with nephrotic syndrome and symptomatic hyperthyroidism due to GD. Kidney biopsy revealed a secondary MN pattern. Immunohistochemical staining for PLA2R was negative, and thyroglobulin showed weak and segmental staining along the glomerular capillary. Anti-thyroid peroxidase (TPO) antibody test was not performed. The patient was treated for GD with methimazole and prednisone, and despite reaching clinical improvement after 8 months, proteinuria remained close to nephrotic levels. In this scenario, the patient was submitted to radioactive iodine, and there was a dramatic reduction in proteinuria levels after treatment. In conclusion, GD association with MN is rare, and when present, diagnosis using PLA2R and immunohistochemistry can be useful in determining association. In addition, radioactive iodine therapy can be an effective treatment modality when preceded with immunosuppressive corticosteroid therapy.
Subject(s)
Glomerulonephritis, Membranous , Graves Disease , Thyroid Neoplasms , Glomerulonephritis, Membranous/diagnosis , Glomerulonephritis, Membranous/drug therapy , Glomerulonephritis, Membranous/etiology , Graves Disease/complications , Graves Disease/diagnosis , Graves Disease/drug therapy , Humans , Iodine Radioisotopes/therapeutic use , Male , Middle Aged , ProteinuriaABSTRACT
The prolonged lockdown of health facilities providing non-urgent gamete cryopreservation-as currently recommended by many reproductive medicine entities and regulatory authorities due to the SARS-CoV-2 pandemic will be detrimental for subgroups of male infertility patients. We believe the existing recommendations should be promptly modified and propose that the same permissive approach for sperm banking granted for men with cancer is expanded to other groups of vulnerable patients. These groups include infertility patients (eg, azoospermic and cryptozoospermic) undergoing medical or surgical treatment to improve sperm quantity and quality, as well as males of reproductive age affected by inflammatory and systemic auto-immune diseases who are about to start treatment with gonadotoxic drugs or who are under remission. In both scenarios, the "fertility window" may be transitory; postponing diagnostic semen analysis and sperm banking in these men could compromise the prospects of biological parenthood. Moreover, we provide recommendations on how to continue the provision of andrological services in a considered manner and a safe environment. Our opinion is timely and relevant given the fact that fertility services are currently rated as of low priority in most countries.
Subject(s)
Andrology/organization & administration , COVID-19 , Health Services Accessibility/organization & administration , Health Services Needs and Demand/organization & administration , Infertility, Male/therapy , Needs Assessment/organization & administration , Humans , Infertility, Male/diagnosis , Infertility, Male/physiopathology , MaleABSTRACT
Damage-associated molecular patterns (DAMPs) are endogenous molecules that are released into the extracellular space under conditions of activation, cellular stress, or tissue damage. These molecules are recognized by pattern-recognition receptors (PRRs) and can induce inflammation and immune responses in the absence of infection. An increasing number of DAMPs have been linked to the pathogenesis of many auto-immune diseases, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), psoriatic arthritis, and systemic sclerosis (SSc); as they promote the maturation/activation of different immune cells and pro-inflammatory cytokines production associated with these diseases. Several studies suggest that the loss of tolerance to self-antigens in these diseases could be due to continuous exposure to DAMPs. Thus, understanding the mechanisms of sterile inflammation triggered by DAMPs is important to elucidate novel therapeutic strategies for the treatment of various auto-immune diseases through inhibition or modulation the expression of these molecules. To this end, this review describes different DAMPs, their molecular characteristics, their modifications, and the receptors through which they activate an immune response while considering their role in the pathogenesis of various auto-immune diseases.
Subject(s)
Autoimmune Diseases/immunology , Lupus Erythematosus, Systemic/immunology , Receptors, Pattern Recognition/metabolism , Animals , Autoimmunity , Cytokines/metabolism , Humans , Immune Tolerance , Inflammation , Signal TransductionABSTRACT
The most recently recognized types of immune cells, the innate lymphoid cells (ILCs), have been sub-divided according to respective distinct expression profiles of regulatory factors or/and cytokines. ILCs have also been shown to participate in a variety of beneficial immune responses, including participation in attack against pathogens and mediation of the pre-inflammatory and inflammatory responses through their production of pro-inflammatory cytokines. As such, while the ILCs exert protective effects they may also become detrimental upon dysregulation. Indeed, recent studies of the ILCs have revealed a strong association with the advent and pathogenesis of several common autoimmune diseases, including psoriasis, inflammatory bowel disease (IBD) and multiple sclerosis (MS). Though the ILCs belong to lineage negative cells that are distinctive from the Th cells, the profiles of secreted cytokines from the ILCs overlap with those of the corresponding Th subsets. Nevertheless, considering that the ILCs belong to the innate immune system and the Th cells belong to the adaptive immune system, it is expected that the ILCs should function at the early stage of diseases and the Th cells should exert predominant effects at the late stage of diseases. Therefore, it is intriguing to consider targeting of ILCs for therapy by targeting the corresponding cytokines at the early stage of diseases, with the late stage cytokine targeting mainly influencing the Th cells' function. Here, we review the knowledge to date on the roles of ILCs in various autoimmune diseases and discuss their potential as new therapeutic targets.
Subject(s)
Autoimmune Diseases/immunology , Cytokines/metabolism , Immunity, Innate , Immunotherapy/methods , Lymphocytes/immunology , Animals , Humans , Th1 Cells/immunology , Th1-Th2 Balance , Th2 Cells/immunologyABSTRACT
A complex interaction of genetic and environmental factors can trigger the immune-mediated mechanism responsible for type 1 diabetes mellitus (T1DM) establishment. Environmental factors may initiate and possibly sustain, accelerate, or retard damage to ß-cells. The role of environmental factors in this process has been exhaustive studied and viruses are among the most probable ones, especially enteroviruses. Improvements in enterovirus detection methods and randomized studies with patient follow-up have confirmed the importance of human enterovirus in the pathogenesis of T1DM. The genetic risk of T1DM and particular innate and acquired immune responses to enterovirus infection contribute to a tolerance to T1DM-related autoantigens. However, the frequency, mechanisms, and pathways of virally induced autoimmunity and ß-cell destruction in T1DM remain to be determined. It is difficult to investigate the role of enterovirus infection in T1DM because of several concomitant mechanisms by which the virus damages pancreatic ß-cells, which, consequently, may lead to T1DM establishment. Advances in molecular and genomic studies may facilitate the identification of pathways at earlier stages of autoimmunity when preventive and therapeutic approaches may be more effective.
ABSTRACT
Introducción: el virus de la Varicela es causante de varias complicaciones en el curso de la misma, entre ellas está la Mielitis transversa. Su mecanismo es autoinmune y se reporta que 30 por ciento de los casos evoluciona de forma crónica con serias secuelas invalidantes.Objetivo: presentar una paciente con diagnóstico de Mielitis transversa aguda como complicación en el curso de la Varicela en el adulto. Presentación del caso: mujer de 45 años sin antecedentes patológicos y con 15 días de evolución con Varicela. Las manifestaciones clínicas neurológicas, el estudio del Líquido Cefalorraquídeo, las imágenes de la Resonancia Magnética Nuclear, la velocidad de conducción nerviosa, la respuesta a la terapéutica impuesta y la evolución apoyaron el diagnóstico.Conclusiones: la Mielitis transversa aguda es una complicación poco frecuente de la Varicela en el adulto. Con un diagnóstico temprano y un tratamiento oportuno la evolución puede ser favorable(AU)
Introduction: chickenpox virus causes several clinical complications during the course of the disease; among them is transverse Myelitis. Its mechanism is auto immune and 30 percent is reported to evolve to a chronic presentation with invalidating consequences. Objective: to show a patient with an acute transverse Myelitis as a complication of the course of the chickenpox in an adult. Case Presentation: we show a 45 years old female, without previous history of a disease and 15 days of evolution of the chickenpox. The clinical neurological manifestations supported by the study of the Cerebrospinal Fluid, the images of the Nuclear Magnetic Resonance , the speed of the nervous conduction, the response to the therapeutics and its evolution support the diagnosis.Conclusions: transverse Myelitis is a non-frequent complication of chickenpox in adults, with an early diagnosis and an appropriate treatment, the evolution could be positive(AU)
Subject(s)
HumansABSTRACT
Co-morbid auto-immune disorders may affect 0.2% of the population. We present the clinical and electrodiagnostic findings of 2 patients with inflammatory bowel disease and myasthenia gravis from a Brazilian cohort of 218 inflammatory bowel disease patients. Patient 1: A 40year-old man was diagnosed with ulcerative colitis at age 37 and underwent total colectomy 3years later. After prednisone was tapered, he experienced a clinical relapse and was diagnosed with Crohn's disease. He then developed quadriparesis, bilateral ptosis, dysphagia and dysarthria. Patient 2: A 41year-old woman (diagnosed with ulcerative colitis and primary sclerosing cholangitis at age 35) developed speech impairment and ptosis. On both patients, symptoms quickly progressed over few weeks. Myasthenia gravis was diagnosed and confirmed by abnormal repetitive nerve stimulation and elevated anti-acetylcholine receptor antibody titers. Pyridostigmine and prednisone successfully treated both patients. Myasthenia gravis prevalence over 9years was 0.9%. Myasthenia gravis clinical course was not significantly modified by inflammatory bowel disease relapses and should be suspected with new onset weakness.
Subject(s)
Inflammatory Bowel Diseases/complications , Myasthenia Gravis/complications , Adult , Brazil/epidemiology , Female , Humans , Inflammatory Bowel Diseases/epidemiology , Male , Myasthenia Gravis/epidemiologyABSTRACT
El presente artículo es el resultado de la investi-gación Afección autoinmune y goce, llevada a cabo en el marco de la Maestría en Investigación psicoanalítica de la Universidad de Antioquia (Colombia). El propósito del mismo es desarrollar uno de los hallazgos de la investigación, según el cual habría una posible articulación entre los enigmas que plantean las afecciones autoinmu-nes y los avatares del deseo, los cuales tendrían un eco en el cuerpo. Lo anterior, partiendo de la concepción psicoanalítica del cuerpo como terri-torio de lo psíquico, como lugar de inscripción de las huellas que se producen en la relación al Otro, en la que se juega lo concerniente al goce y al deseo...
This article is the result of Autoimmune Condition and Jouissance research, carried out as part of the Master in Psychoanalytic Research of the University of Antioquia, Colombia. The purpose is to develop one of the research findings, according to which there would be a possible link between the enigmas posed by autoimmune conditions and the vicissitudes of desire which would have an echo in the body. This is based on the psy-choanalytic idea of the body as a psychic territory, place of the inscription of the traces that occur in the relation to the Other, involving jouissance and desire...
Cet article est le résultat du projet de recherche Maladie auto-immune et jouissance, effectué dans le cadre du Master en recherche psycha-nalytique de L´Université d´Antioquia à Medellín, Colombie. Le but de cet article est donc de déve-lopper lun des résultats de ladite recherche, selon lequel il y aurait une articulation entre les énigmes posées par les maladies auto-immunes et les vicissitudes du désir, lesquelles auraient un écho sur le corps. Cette approche se base sur la conception psychanalytique du corps en tant que territoire du psychisme, comme un lieu dinscription des traces que la relation avec l´Autre produit, où ce qui concerne la jouissance et le désir est en jeu...
Subject(s)
Humans , Psychoanalysis , Sexual Dysfunctions, PsychologicalABSTRACT
Pemphigus vulgaris is a systemic auto-immune medical condition that mainly manifests with changes in skinand vasculopathy. This is a case report of a 69-year-old male with confirmed histopathologic diagnosis of Pemphigus vulgaris presenting ulterior Cognitive Impairment, mostly in executive function. The patient was treated using steroids, immunomodulatory therapy, fluoxetine and galantamine. Neuropsychological testing and magnetic resonance (MRI) were performed. This is the first report of correlational cognitive impairment with Pemphigus vulgaris in the literature. Physicians should be aware of vascular causes for cognitive impairment in patients presenting auto-immune conditions.
Phemphigus vulgaris é uma condição médica sistêmica autoimune que principalmente se manifesta com alterações de pele e vasculopatia. Este é um caso de um homem de 69 anos com diagnóstico histopatológico de Phemphigus vulgaris apresentando posterior comprometimento cognitivo, predominante em funções executivas. O paciente foi tratado com esteroides, terapia imunomoduladora, fluoxetina e galantamina. Avaliação neuropsicológica e ressonância magnética foram realizados. Este é o primeiro relato correlacionando comprometimento cognitivo a pênfigo vulgar na literatura. Os clínicos devem estar cientes das causas vasculares para comprometimento cognitivo em pacientes se apresentando comcondições autoimunes.
Subject(s)
Humans , Autoimmune Diseases , Dementia, Vascular , Dementia , Cognitive Dysfunction , Neuropsychological TestsABSTRACT
Pemphigus vulgaris is a systemic auto-immune medical condition that mainly manifests with changes in skin and vasculopathy. This is a case report of a 69-year-old male with confirmed histopathologic diagnosis of Pemphigus vulgaris presenting ulterior Cognitive Impairment, mostly in executive function. The patient was treated using steroids, immunomodulatory therapy, fluoxetine and galantamine. Neuropsychological testing and magnetic resonance (MRI) were performed. This is the first report of correlational cognitive impairment with Pemphigus vulgaris in the literature. Physicians should be aware of vascular causes for cognitive impairment in patients presenting auto-immune conditions.
Phemphigus vulgaris é uma condição médica sistêmica autoimune que principalmente se manifesta com alterações de pele e vasculopatia. Este é um caso de um homem de 69 anos com diagnóstico histopatológico de Phemphigus vulgaris apresentando posterior comprometimento cognitivo, predominante em funções executivas. O paciente foi tratado com esteroides, terapia imunomoduladora, fluoxetina e galantamina. Avaliação neuropsicológica e ressonância magnética foram realizados. Este é o primeiro relato correlacionando comprometimento cognitivo a pênfigo vulgar na literatura. Os clínicos devem estar cientes das causas vasculares para comprometimento cognitivo em pacientes se apresentando com condições autoimunes.
ABSTRACT
Um cobaio (Caviaporcellus) do sexo feminino, com sete anos de idade e pesando 0,64 kg foi atendido emfunção de um tumor cutâneo isolado, localizado na região tóraco-lombar. O tumor media 4,5 x 3,8 cm, era ulcerado,firme e restrito à epiderme. A massa foi excisada após anestesia geral pela associação de cloridrato decetamina, cloridrato de xilazina e sulfato de atropina. O procedimento cirúrgico e a recuperação da pacienteforam satisfatórios, e as suturas cutâneas foram removidas após oito dias. Macroscopicamente, a superfície decorte do tumor era macia, marrom-acinzentada e levemente lobulada. Microscopicamente, havia proliferaçãode células basais, com muitas áreas de diferenciação folicular, caracterizadas por áreas císticas preenchidaspor ceratina laminada, simulando o terço médio do folículo piloso. Em algumas áreas o estroma do tumor eraseveramente infiltrado por linfócitos, plasmócitos, macrófagos e células gigantes de corpo estranho ao redorde restos de queratina. Tricoepiteliomas são uma forma de diferenciação anexa dos tumores de células basais.Apesar de descritos em cães, e atualmente mesmo em cobaios, sua presença ainda não havia sido relatada em Caviaporcellus em 1995, quando este caso foi originalmente apresentado em um congresso. (AU)
A seven-year old female Guinea Pig (Caviaporcellus) weighing 0.64 kg was examined because of a solitary skin tumor in the toraco-lumbar region. The tumor measured 4.5 by 3.8 cm, was ulcerated, firm and restricted to the epidermis. The mass was excised surgically after general anesthesia with the association ketamine hydrochloride, xylazine hydrochloride and atropine sulfate. The surgical procedureand the recovery were uneventful and the skin sutures were removed after eight days. Grossly, the cutsurface of the tumor was soft, brown-grayish and slightly lobulated. Microscopically, there was proliferationof basal cells with many areas of follicular differentiation, characterized by cystic areas filled with laminated keratin simulating the middle third of the hair follicle. In some areas the stroma of the tumor was severely infiltrated by lymphocytes, plasmacytes, macrophages and foreign body giant cells around keratin remnants. Trichoepitheliomas are a form of adnexal differentiation of basal cell tumors. While they are described in dogs and now even in Guinea Pigs, their presence was not reported in Caviaporcellus in 1995, when this case was originally presented in a congress. (AU)
Subject(s)
Animals , Rodentia , Neoplasms/veterinary , XylazineABSTRACT
O pênfigo foliáceo é uma dermatopatia auto-imune de rara ocorrência na clínica dermatológica felina que acomete gatos adultos a idosos, sem predileção sexual e racial. O diagnóstico é realizado pelo histórico, exame clínico e histopatológico cutâneo. O tratamento consiste no uso de doses imunosupressoras de glicocorticóidesorais e em casos onde este não oferece resultados satisfatórios, o clorambucil pode ser utilizado. O uso daprednisolona na dosagem de 4 mg/kg a cada 24 horas foi eficaz em dois dos casos aqui descritos, sendo essamedicação preconizada na manutenção desses animais. No caso refratário ao tratamento com a prednisolona,instituiu-se o uso da dexametasona (0,2 mg/kg a cada 24 horas) e posteriormente o protocolo terapêutico com o clorambucil (0,1 mg/kg a cada 24 horas),ambos demonstrando pobre resposta terapêutica. O uso do deflazacort na dosagem de 0,3 mg/kg a cada 24 horas foi preconizado com excelente resposta terapêutica neste caso. O presente trabalho tem o objetivo de relatar três casos de pênfigo foliáceo em felinos pela rara ocorrência da doença no Brasil, ressaltando a importância do exame clínico-dermatológico, diagnóstico precoce e tratamento. (AU)
Pemphigus foliaceus is an auto-immune skin disease of rare occurrence in feline dermatology practice that affects middle-aged and older cats. Sex and breed predilections are not noted. The diagnosis is based on the history, clinical examination and histopathology of skin biopsies. The treatment involves oral immunosuppressivedoses of corticosteroids and in cases where it does not give satisfactory results, chlorambucil may be used. The prednisolone use in dosage of 0.2 mg/kg every 24 hours was effective in two of the described cases being successful in the maintenance of these animals. When treating the disease with prednisolone, the dexamethasone dosage was 0.2 mg/kg every 24 hours and afterwards chlorambucil at 0.1 mg/kg every 24 hours, both of them showed poor therapeutic response. The use of deflazacort in dosage of 0.3 mg/kg at 24 hours was successful with excellent therapeutic response. The purpose of the current work is to relate three pemphigus foliaceus cases in felines, a disease which rarely occurs in Brazil. We highlight the importance of the clinical-dermatologic examination, the precocious diagnosis and the treatment. (AU)