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Abstract(s)
Introdução: O Lúpus Eritematoso Sistémico é uma doença autoimune, de curso flutuante, envolvimento multiorgânico e, habitualmente, com evolução crónica. O diagnóstico desta patologia é feito com base nas manifestações clínicas e alterações laboratoriais, e com o auxílio dos critérios de classificação revistos do Colégio Americano de Reumatologia de 1997. No entanto devido à sua grande heterogeneidade sintomatológica, o diagnóstico torna-se frequentemente um desafio. A Nefropatia Lúpica está presente em aproximadamente 50% dos doentes, tendo também um espetro variável de apresentações. A terapêutica farmacológica existente é por vezes pouco satisfatória e está associada a efeitos adversos não desprezíveis. O objetivo deste estudo passa por perceber quais as principais formas de apresentação clínica desta patologia na população da Beira Interior, como se manifesta em termos de nefropatia, e se existe relevância da terapêutica de indução e da presença de antecedentes pessoais ou familiares no curso da doença.
Materiais e métodos: É um estudo observacional de tipo longitudinal e retrospetivo, descritivo e correlacional. Os dados clínicos foram recolhidos num único momento entre janeiro e fevereiro de 2019, através das bases de dados dos serviços de Nefrologia da Unidade Local de Saúde de Castelo Branco e Reumatologia do Centro Hospitalar Universitário Cova da Beira, bem como do programa SClinic. Na análise e tratamento estatístico dos dados investigaram-se as relações entre as variáveis sociodemográficas e epidemiológicas, e caracterizaram-se as formas de apresentação e as manifestações renais ao longo do curso da doença, bem como a terapêutica de indução adotada, através do software Statistical Package for the Social Sciences®, versão 25 para Windows®.
Resultados: A população do estudo é constituída por 37 pacientes, 4 (10,8%) do género masculino e 33 (89,2%) do género feminino, com uma média de idades de 46,33 (±12,57) anos. Os antecedentes pessoais mais prevalentes foram os cardiovasculares (62,2%), os endocrinometabólicos (45,9%), os gastrointestinais (43,2%), e os neuropsiquiátricos (35,1%), enquanto que os antecedentes familiares se mostraram pouco relevantes (10,8%). Relativamente às manifestações iniciais de acordo com os critérios, as mais comuns foram a artrite (69,4%), as manifestações mucocutâneas e imunológicas (ambas com 61,1%), as hematológicas (50,0%) e as alterações do sedimento urinário (38,9%). Ao longo do curso da doença, as alterações nefrológicas mais apresentadas foram também as alterações assintomáticas (59,5%) e a síndrome/proteinúria nefrótica (21,6%). A média do primeiro valor de proteinúria registado foi de 2,832 (± 3,271) gramas nas 24 horas, tendo sido observada uma relação estatisticamente significativa entre o mesmo e a presença de síndrome/proteinúria nefrótica ao longo do curso da doença (p-value=0,033). Dos 19 pacientes com resultado de biópsia renal, a classe IV foi a mais prevalente (n=11). A corticoterapia foi a medida farmacológica mais utilizada (91,4%), seguida pelo micofenolato de mofetil (20,0%). Não existe relação entre as combinações terapêuticas e o número de recidivas registadas. No entanto, foi demonstrada uma relação estatisticamente significativa (p-value=0,048) entre as alterações iniciais do sedimento urinário (segundo os critérios) e o uso de ciclofosfamida.
Conclusões: Os resultados obtidos levam a concluir que esta patologia pode ter um impacto significativo na qualidade de vida, principalmente quando compromete sistemas como o nefrogénico. O seu diagnóstico, tipicamente desafiador, aliado à terapêutica que muitas vezes é insuficiente para o controlo dos sintomas, torna urgente a procura de novas terapêuticas e o estabelecimento de guidelines para orientação diagnóstica e do follow-up dos pacientes.
Introduction: Systemic lupus erythematosus is an autoimmune fluctuating disease, with multiorganic involvement and usually chronic evolution. The diagnosis is based on clinical expressions, laboratory abnormalities, and with the aid of the revised classification criteria of the American College of Rheumatology of 1997. However, due to its great symptomatologic heterogeneity, diagnosis often becomes a challenge. Lupus Nephropathy is present in approximately 50% of patients, and also has a variety of appearances. The pharmacological therapy available is typically unsatisfactory and associated with undesirable side effects. The purpose of this study is to understand the main types of clinical expressions in the population of Beira Interior, how the disease manifests in terms of nephropathy, and whether there is relevance of the induction therapy or the personal and family history in the course of disease. Materials and methods: This is an observational study that is longitudinal and retrospective, descriptive and correlational. The clinical data was collected on a single occasion between January and February of 2019, supported by the databases of the services of Nephrology of the Local Health Unit of Castelo Branco and Rheumatology of the University Hospital Center of Cova da Beira, as well as the SClinic program. In the analysis and statistical treatment of the data, the relationships between the sociodemographic and epidemiological variables were investigated. The forms of presentation and renal manifestation throughout the course of the disease were also characterized, as well as the induction therapy adopted through Statistical Package for the Social Sciences® software, version 25 for Windows®. Results: The study population consists of 37 patients, 4 (10,8%) males and 33 (89,2%) females, with an average of 46,33 (± 12,57) years of age. The most prevalent pre-existing disorders were cardiovascular (62,2%), endocrine metabolic (45,9%), gastrointestinal (43,2%), and of neuropsychiatric origin (35,1%), while family history was shown to be irrelevant (10,8%). The most frequent initial expressions according to the criteria were arthritis (69,4%), mucocutaneous (61,1%), immunological (61,1%), and haematological manifestations (50,0%), and in addition, alterations in urinary sediment (38,9%). During the course of the disease, the most nephrological alterations presented were also asymptomatic alterations (59,5%) and nephrotic syndrome/proteinuria (21,6%). The mean of the first recorded value of proteinuria was 2,832 (± 3,271) grams at 24 hours. A statistically significant relationship was observed between the value of proteinuria and the presence of nephrotic syndrome/proteinuria over the course of the disease (p-value=0,033). Of the 19 patients with renal biopsy results, class IV was the most prevalent (n=11). Corticosteroids were the most used pharmacological treatment (91,4%), followed by mycophenolate mofetil (20,0%). There was no relationship between the therapeutic combinations and the number of recurrences recorded. However, statistically significant relation (p-value=0,048) was demonstrated between the initial changes of the urinary sediment (according to the criteria) and the use of cyclophosphamide. Conclusions: The results obtained lead to the conclusion that this pathology can have a significant impact on the quality of life, especially when it compromises systems such as the nephrogenic system. The diagnosis that is typically challenging, together with therapy that is often insufficient to control the symptoms, makes it urgent to seek new therapies and establish guidelines for the diagnosis and the follow-up of patients.
Introduction: Systemic lupus erythematosus is an autoimmune fluctuating disease, with multiorganic involvement and usually chronic evolution. The diagnosis is based on clinical expressions, laboratory abnormalities, and with the aid of the revised classification criteria of the American College of Rheumatology of 1997. However, due to its great symptomatologic heterogeneity, diagnosis often becomes a challenge. Lupus Nephropathy is present in approximately 50% of patients, and also has a variety of appearances. The pharmacological therapy available is typically unsatisfactory and associated with undesirable side effects. The purpose of this study is to understand the main types of clinical expressions in the population of Beira Interior, how the disease manifests in terms of nephropathy, and whether there is relevance of the induction therapy or the personal and family history in the course of disease. Materials and methods: This is an observational study that is longitudinal and retrospective, descriptive and correlational. The clinical data was collected on a single occasion between January and February of 2019, supported by the databases of the services of Nephrology of the Local Health Unit of Castelo Branco and Rheumatology of the University Hospital Center of Cova da Beira, as well as the SClinic program. In the analysis and statistical treatment of the data, the relationships between the sociodemographic and epidemiological variables were investigated. The forms of presentation and renal manifestation throughout the course of the disease were also characterized, as well as the induction therapy adopted through Statistical Package for the Social Sciences® software, version 25 for Windows®. Results: The study population consists of 37 patients, 4 (10,8%) males and 33 (89,2%) females, with an average of 46,33 (± 12,57) years of age. The most prevalent pre-existing disorders were cardiovascular (62,2%), endocrine metabolic (45,9%), gastrointestinal (43,2%), and of neuropsychiatric origin (35,1%), while family history was shown to be irrelevant (10,8%). The most frequent initial expressions according to the criteria were arthritis (69,4%), mucocutaneous (61,1%), immunological (61,1%), and haematological manifestations (50,0%), and in addition, alterations in urinary sediment (38,9%). During the course of the disease, the most nephrological alterations presented were also asymptomatic alterations (59,5%) and nephrotic syndrome/proteinuria (21,6%). The mean of the first recorded value of proteinuria was 2,832 (± 3,271) grams at 24 hours. A statistically significant relationship was observed between the value of proteinuria and the presence of nephrotic syndrome/proteinuria over the course of the disease (p-value=0,033). Of the 19 patients with renal biopsy results, class IV was the most prevalent (n=11). Corticosteroids were the most used pharmacological treatment (91,4%), followed by mycophenolate mofetil (20,0%). There was no relationship between the therapeutic combinations and the number of recurrences recorded. However, statistically significant relation (p-value=0,048) was demonstrated between the initial changes of the urinary sediment (according to the criteria) and the use of cyclophosphamide. Conclusions: The results obtained lead to the conclusion that this pathology can have a significant impact on the quality of life, especially when it compromises systems such as the nephrogenic system. The diagnosis that is typically challenging, together with therapy that is often insufficient to control the symptoms, makes it urgent to seek new therapies and establish guidelines for the diagnosis and the follow-up of patients.
Description
Keywords
Antecedentes Pessoais e Familiares Diagnóstico Lúpus Eritematoso Sistémico Nefropatia Lúpica Terapêutica de Indução