RESUMO
Systemic lupus erythematosus (SLE) is an idiopathic chronic autoimmune disease that can affect any organ in the body, including the neurological system. Multiple factors, such as environmental (infections), genetic (many HLA alleles including DR2 and DR3, and genes including C4), and immunological influences on self-antigens, such as nuclear antigens, lead to the formation of multiple autoantibodies that cause deleterious damage to bodily tissues and organs. The production of autoantibodies, such as anti-dsDNA, anti-SS(A), anti-SS(B), anti-Smith, and anti-neuronal DNA are characteristic features of this disease. This autoimmune disease results from a failure of the mechanisms responsible for maintaining self-tolerance in T cells, B cells, or both. Immune complexes, circulating antibodies, cytokines, and autoreactive T lymphocytes are responsible for tissue injury in this autoimmune disease. The diagnosis of SLE is a rheumatological challenge despite the availability of clinical criteria. NPSLE was previously referred to as lupus cerebritis or lupus sclerosis. However, these terms are no longer recommended because there is no definitive pathological cause for the neuropsychiatric manifestations of SLE. Currently, the treatment options are primarily based on symptomatic presentations. These include the use of antipsychotics, antidepressants, and anxiolytic medications for the treatment of psychiatric and mood disorders. Antiepileptic drugs to treat seizures, and immunosuppressants (e.g., corticosteroids, azathioprine, and mycophenolate mofetil), are directed against inflammatory responses along with non-pharmacological interventions.
Assuntos
Lúpus Eritematoso Sistêmico , Vasculite Associada ao Lúpus do Sistema Nervoso Central , Humanos , Vasculite Associada ao Lúpus do Sistema Nervoso Central/diagnóstico , Vasculite Associada ao Lúpus do Sistema Nervoso Central/tratamento farmacológico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/genética , Autoanticorpos , Imunossupressores/uso terapêutico , Convulsões/tratamento farmacológicoRESUMO
Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by a defect in the phagocytic function of the innate immune system owing to mutations in genes encoding the five subunits of the nicotinamide adenine dinucleotide phosphatase (NADPH) oxidase enzyme complex. This review aimed to provide a comprehensive approach to the pathogens associated with chronic granulomatous disease (CGD) and its management. Patients with CGD, often children, have recurrent life-threatening infections and may develop infectious or inflammatory complications. The most common microorganisms observed in the patients with CGD are Staphylococcus aureus, Aspergillus spp., Candida spp., Nocardia spp., Burkholderia spp., Serratia spp., and Salmonella spp. Antibacterial prophylaxis with trimethoprim-sulfamethoxazole, antifungal prophylaxis usually with itraconazole, and interferon gamma immunotherapy have been successfully used in reducing infection in CGD. Haematopoietic stem cell transplantation (HCT) have been successfully proven to be the treatment of choice in patients with CGD.
RESUMO
Transient hypogammaglobulinemia of infancy (THI) is a primary immunodeficiency caused by a temporary decline in serum immunoglobulin G (IgG) levels greater than two standard deviations below the mean age-specific reference values in infants between 5 and 24 months of age. Preterm infants are particularly susceptible to THI, as IgG is only transferred across the placenta from mother to infant during the third trimester of pregnancy. This study aimed to conduct a systematic review of the diagnostic criteria for transient hypogammaglobulinemia of infancy. Systematic review: Three electronic databases (PubMed, MEDLINE, and Google Scholar) were manually searched from September 2021 to April 2022. Abstracts were screened to assess their fit to the inclusion criteria. Data were extracted from the selected studies using an adapted extraction tool (Cochrane). The studies were then assessed for bias using an assessment tool adapted from Cochrane. Of the 215 identified articles, 16 were eligible for examining the diagnostic criteria of THI. These studies were also assessed for bias in the six domains. A total of five studies (31%) had a low risk of bias, while four studies (25%) had a high risk of bias, and bias in the case of seven studies (44%) was unclear. We conclude that THI is only definitively diagnosed after abnormal IgG levels normalise. Hence, THI is not a benign condition, and monitoring for subsequent recurrent infections must be conducted. The diagnostic criteria should also include vaccine and isohaemagglutinin responses to differentiate THI from other immunological disorders in infants.
RESUMO
Severe combined immunodeficiency (SCID) is a primary inherited immunodeficiency disease that presents before the age of three months and can be fatal. It is usually due to opportunistic infections caused by bacteria, viruses, fungi, and protozoa resulting in a decrease in number and impairment in the function of T and B cells. Autosomal, X-linked, and sporadic forms exist. Evidence of recurrent opportunistic infections and lymphopenia very early in life should prompt immunological investigation and suspicion of this rare disorder. Adequate stem cell transplantation is the treatment of choice. This review aimed to provide a comprehensive approach to the microorganisms associated with severe combined immunodeficiency (SCID) and its management. We describe SCID as a syndrome and summarize the different microorganisms that affect children and how they can be investigated and treated.
RESUMO
OBJECTIVE: This study was undertaken to determine the prevalence of human immunodeficiency virus-type 1 (HIV-1) infection in patients with pulmonary tuberculosis at the National Chest Hospital in Jamaica. METHODS: This retrospective study reviewed the hospital records of 537 patients admitted over a seven-year period from 1995 to 2001. We used a standardized data collection form to obtain data for sociodemographic characteristics, clinical features, signs and symptoms, laboratory diagnosis, treatment and outcome. RESULTS: We found that 11.6% (47/406) of the patients who met the inclusion criteria and were diagnosed as having pulmonary tuberculosis were HIV-1 seropositive. Most HIV-positive patients with tuberculosis were males, and prevalence of HIV coinfection among patients with tuberculosis was highest in patients aged 30-39 years. The mortality rate in patients with tuberculosis and HIV infection was 23.4% (11/47) compared to 3.9% (14/359; P = 0.001) in HIV-negative patients. Patients were treated with standard quadruple drug therapy. No multiple drug resistance was noted in the Mycobacterium tuberculosis isolates. CONCLUSIONS: The prevalence of HIV in patients with tuberculosis in Jamaica is similar to that in other developing countries, but the mortality rate is higher and this warrants prompt diagnosis of HIV infection and early institution of highly active antiretroviral therapy.
Assuntos
Infecções por HIV/epidemiologia , HIV-1 , Tuberculose Pulmonar/epidemiologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Antituberculosos/farmacologia , Antituberculosos/uso terapêutico , Criança , Pré-Escolar , Comorbidade , Estudos Transversais , Interpretação Estatística de Dados , Feminino , Soronegatividade para HIV , Soropositividade para HIV/epidemiologia , Soropositividade para HIV/mortalidade , HIV-1/imunologia , Hospitais Especializados , Humanos , Jamaica/epidemiologia , Masculino , Pessoa de Meia-Idade , Mycobacterium tuberculosis/efeitos dos fármacos , Mycobacterium tuberculosis/isolamento & purificação , Estudos Retrospectivos , Fatores Sexuais , Escarro/microbiologia , Tuberculose Pulmonar/tratamento farmacológico , Tuberculose Pulmonar/mortalidadeRESUMO
Objective. This study was undertaken to determine the prevalence of human immunodeficiency virus-type 1 (HIV-1) infection in patients with pulmonary tuberculosis at the National Chest Hospital in Jamaica. Methods. This retrospective study reviewed the hospital records of 537 patients admitted over a seven-year period from 1995 to 2001. We used a standardized data collection form to obtain data for sociodemographic characteristics, clinical features, signs and symptoms, laboratory diagnosis, treatment and outcome. Results. We found that 11.6% (47/406) of the patients who met the inclusion criteria and were diagnosed as having pulmonary tuberculosis were HIV-1 seropositive. Most HIV-positive patients with tuberculosis were males, and prevalence of HIV coinfection among patients with tuberculosis was highest in patients aged 30-39 years. The mortality rate in patients with tuberculosis and HIV infection was 23.4% (11/47) compared to 3.9% (14/359; P = 0.001) in HIVnegative patients. Patients were treated with standard quadruple drug therapy. No multiple drug resistance was noted in the Mycobacterium tuberculosis isolates. Conclusions. The prevalence of HIV in patients with tuberculosis in Jamaica is similar to that in other developing countries, but the mortality rate is higher and this warrants prompt diagnosis of HIV infection and early institution of highly active antiretroviral therapy.
OBJECTIVE: El presente estudio se efectuó para determinar la prevalencia del virus de la inmunodeficiencia humana tipo 1 (VIH-1) en pacientes con tuberculosis pulmonar del National Chest Hospital en Jamaica. MÉTODOS: En este estudio retrospectivo se revisaron los expedientes hospitalarios de 537 pacientes ingresados a lo largo de un período de siete años, de 1995 a 2001. Utili- zamos un formulario uniformado para obtener los datos relacionados con las características sociodemográficas; los rasgos, signos y síntomas clínicos; el diagnóstico de laboratorio; el tratamiento administrado, y los resultados observados. RESULTADOS: Encontramos que 11,6% (47/406) de los pacientes que satisfacían los criterios de inclusión y a quienes se les había diagnosticado tuberculosis pulmonar tenían seropositividad al VIH-1. La mayoría de los pacientes tuberculosos con positividad a VIH eran de sexo masculino, y la mayor prevalencia de infección simultánea con VIH en pacientes tuberculosos se observó en personas entre los 30 y 39 años de edad. La tasa de mortalidad en pacientes con tuberculosis e infección por VIH fue de 23,4% (11/47), en comparación con 3,9% (14/359; P = 0,001) en pacientes sin infección por VIH. A los pacientes se les administró el tratamiento estándar con cuatro medicamentos. No se observó ninguna resistencia en las cepas aisladas de Mycobacterium tuberculosis. CONCLUSIONES: En Jamaica la prevalencia de VIH en pacientes con tuberculosis es parecida a la observada en otros países en desarrollo, pero la tasa de mortalidad en estos pacientes es mayor. Por lo tanto, es imprescindible diagnosticar la infección por VIH en etapa temprana e iniciar de inmediato el tratamiento antirretrovírico de gran actividad.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , HIV-1 , Infecções por HIV/epidemiologia , Tuberculose Pulmonar/epidemiologia , HIV-1 , Fatores Etários , Antituberculosos/farmacologia , Antituberculosos/uso terapêutico , Comorbidade , Estudos Transversais , Interpretação Estatística de Dados , Soronegatividade para HIV , Soropositividade para HIV/epidemiologia , Soropositividade para HIV/mortalidade , Hospitais Especializados , Jamaica/epidemiologia , Mycobacterium tuberculosis/efeitos dos fármacos , Mycobacterium tuberculosis/isolamento & purificação , Estudos Retrospectivos , Fatores Sexuais , Escarro/microbiologia , Tuberculose Pulmonar/tratamento farmacológico , Tuberculose Pulmonar/mortalidadeRESUMO
OBJECTIVE: This study was undertaken to determine the prevalence of human immunodeficiency virus-type 1 (HIV-1) infection in patients with pulmonary tuberculosis at the National Chest Hospital in Jamaica. METHODS: This retrospective study reviewed the hospital records of 537 patients admitted over a seven-year period from 1995 to 2001. We used a standardized data collection form to obtain data for sociodemographic characteristics, clinical features, signs and symptoms, laboratory diagnosis, treatment and outcome. RESULTS: We found that 11.6 percent (47/406) of the patients who met the inclusion criteria and were diagnosed as having pulmonary tuberculosis were HIV-1 seropositive. Most HIV-positive patients with tuberculosis were males, and prevalence of HIV coinfection among patients with tuberculosis was highest in patients aged 30-39 years. The mortality rate in patients with tuberculosis and HIV infection was 23.4 percent (11/47) compared to 3.9 percent (14/359;P=0.001) in HIV-negative patients. Patients were treated with standard quadruple drug therapy. No multiple drug resistance was noted in Mycobacterium tuberculosis isolates. CONCLUSIONS: The prevalence of HIV in patients with tuberculosis in Jamaica is similar to that in other developing countries, but the mortality rate is higher and this warrants prompt diagnosis of HIV infection and early institution of highly active antiretroviral therapy (AU)