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Abstract(s)
A Síndrome de Sjögren é considerada a segunda doença reumática autoimune mais frequente, afetando entre 0,05% e 0,4% da população mundial. Esta doença caracteriza-se pela inflamação crónica das glândulas salivares e lacrimais, dando origem aos seus principais sintomas: xerostomia (boca seca) e xeroftalmia (olho seco), respetivamente. Esta síndrome pode existir isoladamente (Síndrome de Sjögren primário) ou associada a uma doença do tecido conjuntivo como a Artrite Reumatóide ou Lúpus Eritematoso Sistémico (Síndrome de Sjögren secundário).
Apesar da forma primária não estar associada a nenhuma doença do tecido conjuntivo, pode ter sintomas extraglandulares como manifestações músculo-esqueléticas, pulmonares, cutâneas. Estas manifestações fazem, muitas vezes, com que a doença seja subdiagnosticada, uma vez que a sua apresentação pode mimetizar outras doenças, sendo, portanto, necessário um diagnóstico diferencial cuidadoso.
Um dos problemas que sempre surgiu foi o diagnóstico desta doença. Devido à sua complexidade, foram surgindo, ao longo dos anos, diferentes critérios de classificação para a diagnosticar. Estes foram sofrendo reavaliações e reformulações devido à falta de sensibilidade/ especificidade de alguns componentes ou características e devido também ao peso que cada componente deveria ter no diagnóstico desta doença.
Recentemente, foram apresentados os novos critérios de classificação para a Síndrome de Sjögren, sendo um dos mais importantes e de maior sensibilidade e especificidade: a biópsia das glândulas salivares minor.
A Biópsia das glândulas salivares minor é o critério que faz a confirmação do diagnóstico da Síndrome de Sjögren quando há casos suspeitos de terem a doença, sendo também crucial no prognóstico da mesma.
O objetivo deste estudo é demonstrar a importância e precisão da biópsia das glândulas salivares minor no diagnóstico da Síndrome de Sjögren. Neste contexto, vão também ser descritos os principais sintomas e sinais que os casos suspeitos apresentam, outros testes feitos no âmbito da disfunção glandular, perfil imunológico dos casos e a relevância que cada um destes pontos tem no diagnóstico desta doença.
Sjögren’s syndrome is considered the second most frequent autoimmune rheumatic disease, affecting between 0,05% and 0,4% of the world population. The disease is characterized by the inflamation of salivary and lacrimal glands, which are the source of the two main symptoms: xerostomia (meaning dry mouth) and xerophtalmia (meaning dry eyes), respectively. This syndrome exists alone (primary Sjögren’s Syndrome) or in association with a connective tissue disease, like Rheumatoid Arthritis or Systemic Lupus Erythematosus (secondary Sjögren’s Syndrome). Although the primary form is not associated with any connective tissue disease, it can have extraglandular symptoms like musculoskeletal, pulmonary, cutaneous manifestations. Frequently, this manifestations cause the disease to be subdiagnosed, because its presentation can mimetize other diseases. Thus, a careful differential diagnosis is required. It has always been a problem to diagnose correctly this disease. Because of its complexity, over the years have emerged different classification criteria for the diagnosis. This criteria has been suffering revaluations and reformulations thanks to the lack of sensibility/specificity of some components or characteristics and also due to the ponderosity that each component should have in the diagnosis of the disease. Recently new classification criteria were presented for Sjögren Syndrome, being the most important and the most sensitive and specific one: the Labial Salivary Gland Biopsy. The Labial Salivary Gland Biopsy is the criteria that makes the confirmation of the Sjögren Syndrome’s diagnosis when there are suspected cases of having the disease, being also crucial in its prognosis. The aim of this study is to demonstrate the importance and precision of the labial salivary gland biopsy in the diagnosis of Sjögren Syndrome. In this context, it will be described the main symptoms and signs that the suspected cases present, other tests made in the context of glandular dysfunction, immunological profile of the cases and the relevance of each of this points in diagnosis of the disease.
Sjögren’s syndrome is considered the second most frequent autoimmune rheumatic disease, affecting between 0,05% and 0,4% of the world population. The disease is characterized by the inflamation of salivary and lacrimal glands, which are the source of the two main symptoms: xerostomia (meaning dry mouth) and xerophtalmia (meaning dry eyes), respectively. This syndrome exists alone (primary Sjögren’s Syndrome) or in association with a connective tissue disease, like Rheumatoid Arthritis or Systemic Lupus Erythematosus (secondary Sjögren’s Syndrome). Although the primary form is not associated with any connective tissue disease, it can have extraglandular symptoms like musculoskeletal, pulmonary, cutaneous manifestations. Frequently, this manifestations cause the disease to be subdiagnosed, because its presentation can mimetize other diseases. Thus, a careful differential diagnosis is required. It has always been a problem to diagnose correctly this disease. Because of its complexity, over the years have emerged different classification criteria for the diagnosis. This criteria has been suffering revaluations and reformulations thanks to the lack of sensibility/specificity of some components or characteristics and also due to the ponderosity that each component should have in the diagnosis of the disease. Recently new classification criteria were presented for Sjögren Syndrome, being the most important and the most sensitive and specific one: the Labial Salivary Gland Biopsy. The Labial Salivary Gland Biopsy is the criteria that makes the confirmation of the Sjögren Syndrome’s diagnosis when there are suspected cases of having the disease, being also crucial in its prognosis. The aim of this study is to demonstrate the importance and precision of the labial salivary gland biopsy in the diagnosis of Sjögren Syndrome. In this context, it will be described the main symptoms and signs that the suspected cases present, other tests made in the context of glandular dysfunction, immunological profile of the cases and the relevance of each of this points in diagnosis of the disease.
Description
Keywords
Biópsia de Glândulas Salivares Minor Critérios de Diagnóstico Síndrome de Sjögren Síndrome de Sjögren Primário Xerostomia