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Authors
Abstract(s)
Introdução: A pneumoconiose representa uma doença profissional resultante da inalação e da
deposição de pó de minerais e de outras substâncias inorgânicas no pulmão.
Pode ser dividida em diferentes categorias pelo tipo de partículas envolvidas ou pelo tipo de
reação desencadeada. O carvão, a sílica, o asbesto e o talco provocam uma resposta inflamatória
e fibrogénica enquanto o berílio e o cobalto promovem respetivamente uma resposta
granulomatosa e associada a pneumonia de células gigantes. Já o ferro, o estanho e o bário são
consideradas partículas benignas ou inertes.
A silicose resulta da inalação de pequenas partículas de silício na forma de sílica “livre” cristalina
(geralmente quartzo) ou eventualmente de silicatos, minerais que contêm dióxido de silício ligado
a outros minerais como o talco.
Tipicamente manifesta-se após exposição prolongada e caracteriza-se pelo desenvolvimento de
nódulos pulmonares fibróticos, que na forma complicada de doença coalescem formando maciços
fibróticos, com progressão para dispneia, hipoxemia, hipertensão pulmonar e insuficiência
respiratória.
A ocorrência de doenças autoimunes como a esclerose sistémica, a artrite reumatoide, o lúpus
eritematoso sistémico, a vasculite sistémica e renal e a síndrome de Sjögren é maior em indivíduos
expostos à sílica (com ou sem silicose), com um risco médio cinco vezes superior ao risco da
população de indivíduos não expostos.
Especificamente, a síndrome de Sjögren distingue-se pela infiltração linfocítica de glândulas
exócrinas e de outros órgãos, cursando com manifestações glandulares, frequentemente
xeroftalmia e xerostomia, e manifestações extraglandulares como artralgia, artrite, linfadenopatia
generalizada, fenómeno de Raynaud, envolvimento pulmonar intersticial e vasculite.
Objetivos: Atendendo ao facto de a silicose ser reconhecida como a principal causa de invalidez
entre as doenças respiratórias ocupacionais, o presente trabalho tem como primeiro objetivo a
integração dos conhecimentos mais recentes e relevantes acerca dos mecanismos etiológicos e
patofisiológicos envolvidos e ainda da heterogeneidade clínico-patológica e das hipóteses
terapêuticas de que se dispõe atualmente, de modo a desenvolver melhores meios de prevenção e
diagnóstico, bem como terapêuticas mais eficazes. O segundo propósito do trabalho diz respeito à identificação e ao estudo de casos referentes à
coexistência de silicose e de doenças autoimunes, especificamente a síndrome de Sjögren, num
mesmo doente.
O terceiro e último objetivo prende-se com a compreensão da correlação entre os fatores
ambientais e a desregulação autoimune, neste caso em particular da ativação crónica das células
T pela exposição à sílica.
Materiais e métodos: A revisão sistemática baseou-se na leitura integral de artigos presentes
em várias bases de dados: PubMed, Academic Search Complete, MEDLINE Complete, Cochrane
Central Register of Controlled Trials, Cochrane Database of Systematic Reviews e Cochrane
Methodology Register, Cochrane Clinical Answers.
Das publicações obtidas, tendo presente os objetivos do trabalho, foram selecionados 13 artigos.
Para além da análise dos artigos, foi investigada a possibilidade de um caso de silicose associado
a uma doença autoimune no Centro Hospitalar Universitário Cova da Beira (CHCUB), na Covilhã.
Foi apurado apenas um caso de um indivíduo do sexo masculino, com início no ano de 2022,
tendo sido recolhida toda a informação clínica relevante para o trabalho.
Conclusão: Conclui-se assim que a forma crónica da silicose pode influenciar negativamente a
regulação do sistema imunitário, associando-se a doenças autoimunes – esclerose sistémica,
lúpus eritematoso sistémico e, menos frequentemente, síndrome de Sjögren.
Tendo em conta os estudos realizados até ao momento, o rastreio de sinais e de sintomas
característicos das doenças autoimunes supracitadas, neste caso em particular da síndrome
Sjögren, deveria ser considerado nos indivíduos expostos a partículas de minerais. Numa mesma
linha de pensamento, a exposição individual e a ocupação profissional deveriam ser ponderadas
nos indivíduos com diagnóstico prévio de uma ou mais doenças autoimunes.
Sendo a silicose uma doença profissional, seria igualmente importante apostar na divulgação e
implementação de medidas preventivas.
Introduction: Pneumoconiosis represents an occupational disease resulting from inhalation and deposition of mineral dust and other inorganic substances in the lung. It can be divided into different categories by the type of particles involved or the type of reaction triggered. Coal, silica, asbestos and talc provoke an inflammatory and fibrogenic response while beryllium and cobalt respectively promote a granulomatous and giant cell pneumonia-associated response. Iron, tin and barium are considered benign or inert particles. Silicosis results from the inhalation of small particles of silicon in the form of "free" crystalline silica (usually quartz) or possibly silicates, minerals that contain silicon dioxide bound to other minerals such as talc. It typically manifests after prolonged exposure and is characterised by the development of fibrotic pulmonary nodules, which in the complicated form of the disease coalesce to form fibrotic masses, with progression to dyspnoea, hypoxaemia, pulmonary hypertension and respiratory failure. The occurrence of autoimmune diseases such as systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus, systemic and renal vasculitis, and Sjögren's syndrome is higher in individuals exposed to silica (with or without silicosis), with an average risk five times higher than in the population of unexposed individuals. Specifically, Sjögren's syndrome is distinguished by lymphocytic infiltration of exocrine glands and other organs, with glandular manifestations, often xerophthalmia and xerostomia, and extraglandular manifestations such as arthralgia, arthritis, generalised lymphadenopathy, Raynaud's phenomenon, interstitial pulmonary involvement, and vasculitis. Objectives: Since silicosis is recognised as the leading cause of disability among occupational respiratory diseases, this project's first aim is the integration of the most recent and relevant knowledge about the etiological and pathophysiological mechanisms involved as well as the clinical and pathological heterogeneity and the therapeutic options currently available to develop better means of prevention and diagnosis as well as more effective therapies. The second objective of the work is related to the identification and study of cases of coexistence of silicosis and autoimmune diseases, specifically Sjögren's syndrome, in the same patient. The third and last objective is related to the understanding of the correlation between environmental factors and autoimmune dysregulation, in this case in particular the chronic activation of T cells by exposure to silica. Materials and methods: The systematic review was based on the full-text reading of articles from several databases: PubMed, Academic Search Complete, MEDLINE Complete, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews and Cochrane Methodology Register, Cochrane Clinical Answers. From the publications obtained, bearing in mind the objectives of the study, 13 articles were selected. In addition to the analysis of the articles, the possibility of a case of silicosis associated with an autoimmune disease at the Centro Hospitalar Universitário Cova da Beira in Covilhã, was investigated. Only one case of a male individual was found, beginning in 2022, and all clinical information relevant to the work was collected. Conclusion: We conclude that the chronic form of silicosis may negatively influence the regulation of the immune system, being associated with autoimmune diseases - systemic sclerosis, systemic lupus erythematosus and, less frequently, Sjögren's syndrome. In view of the studies carried out to date, screening for signs and symptoms characteristic of the above-mentioned autoimmune diseases, in this particular case Sjögren's syndrome, should be considered in individuals exposed to mineral particles. Along the same line of thought, individual exposure and occupation should be considered in individuals previously diagnosed with one or more autoimmune diseases. As silicosis is an occupational disease, it would be equally important to focus on the dissemination and implementation of preventive measures.
Introduction: Pneumoconiosis represents an occupational disease resulting from inhalation and deposition of mineral dust and other inorganic substances in the lung. It can be divided into different categories by the type of particles involved or the type of reaction triggered. Coal, silica, asbestos and talc provoke an inflammatory and fibrogenic response while beryllium and cobalt respectively promote a granulomatous and giant cell pneumonia-associated response. Iron, tin and barium are considered benign or inert particles. Silicosis results from the inhalation of small particles of silicon in the form of "free" crystalline silica (usually quartz) or possibly silicates, minerals that contain silicon dioxide bound to other minerals such as talc. It typically manifests after prolonged exposure and is characterised by the development of fibrotic pulmonary nodules, which in the complicated form of the disease coalesce to form fibrotic masses, with progression to dyspnoea, hypoxaemia, pulmonary hypertension and respiratory failure. The occurrence of autoimmune diseases such as systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus, systemic and renal vasculitis, and Sjögren's syndrome is higher in individuals exposed to silica (with or without silicosis), with an average risk five times higher than in the population of unexposed individuals. Specifically, Sjögren's syndrome is distinguished by lymphocytic infiltration of exocrine glands and other organs, with glandular manifestations, often xerophthalmia and xerostomia, and extraglandular manifestations such as arthralgia, arthritis, generalised lymphadenopathy, Raynaud's phenomenon, interstitial pulmonary involvement, and vasculitis. Objectives: Since silicosis is recognised as the leading cause of disability among occupational respiratory diseases, this project's first aim is the integration of the most recent and relevant knowledge about the etiological and pathophysiological mechanisms involved as well as the clinical and pathological heterogeneity and the therapeutic options currently available to develop better means of prevention and diagnosis as well as more effective therapies. The second objective of the work is related to the identification and study of cases of coexistence of silicosis and autoimmune diseases, specifically Sjögren's syndrome, in the same patient. The third and last objective is related to the understanding of the correlation between environmental factors and autoimmune dysregulation, in this case in particular the chronic activation of T cells by exposure to silica. Materials and methods: The systematic review was based on the full-text reading of articles from several databases: PubMed, Academic Search Complete, MEDLINE Complete, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews and Cochrane Methodology Register, Cochrane Clinical Answers. From the publications obtained, bearing in mind the objectives of the study, 13 articles were selected. In addition to the analysis of the articles, the possibility of a case of silicosis associated with an autoimmune disease at the Centro Hospitalar Universitário Cova da Beira in Covilhã, was investigated. Only one case of a male individual was found, beginning in 2022, and all clinical information relevant to the work was collected. Conclusion: We conclude that the chronic form of silicosis may negatively influence the regulation of the immune system, being associated with autoimmune diseases - systemic sclerosis, systemic lupus erythematosus and, less frequently, Sjögren's syndrome. In view of the studies carried out to date, screening for signs and symptoms characteristic of the above-mentioned autoimmune diseases, in this particular case Sjögren's syndrome, should be considered in individuals exposed to mineral particles. Along the same line of thought, individual exposure and occupation should be considered in individuals previously diagnosed with one or more autoimmune diseases. As silicosis is an occupational disease, it would be equally important to focus on the dissemination and implementation of preventive measures.
Description
Keywords
Autoimunidade Doenças Ocupacionais Silicose Síndrome de Sjögren