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Características imunopatológicas da doença relacionada à IgG4 com envolvimento oral e maxilofacial
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A doença relacionada à imunoglobulina G4 (IgG4-RD) é uma condição fibroinflamatória sistémica caracterizada por infiltração linfoplasmocitária rica em plasmócitos IgG4+, fibrose estoriforme e elevação variável dos níveis séricos de IgG4. Embora a IgG4-RD seja reconhecida sobretudo em órgãos como pâncreas, rins e glândulas lacrimais, o envolvimento da cavidade oral e região maxilofacial tem sido progressivamente descrito.
A sua apresentação clínica heterogénea e a sobreposição de características com diversas patologias de natureza autoimune, inflamatória e neoplásica tornam a IgG4-RD um desafio diagnóstico relevante em medicina dentária. O crescente conhecimento dos mecanismos imunopatológicos e das alterações histológicas associadas à doença tem permitido compreender melhor a fisiopatologia da doença e a sua expressão nos tecidos orais. Entre as manifestações mais frequentemente descritas incluem-se o aumento das glândulas salivares, xerostomia, tumefações pseudotumorais, envolvimento do palato e alterações da mucosa que dificultam a diferenciação com outras entidades como a Síndrome de Sjögren (SS), sialoadenites crónicas e linfomas.
A presente revisão narrativa tem como objetivo sintetizar o conhecimento atual sobre as características imunopatológicas da IgG4-RD com envolvimento oral e maxilofacial a fim de diminuir as dificuldades inerentes ao diagnóstico precoce desta doença. A discussão das implicações clínicas e abordagens terapêuticas visa contribuir para um melhor conhecimento da IgG4-RD, sublinhando a importância do encaminhamento interdisciplinar e da integração do médico dentista no processo de diagnóstico, de modo a assegurar uma intervenção atempada e adequada.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and variable elevations in serum IgG4 levels. Although IgG4-RD is most commonly recognized in organs such as the pancreas, kidneys, and lacrimal glands, involvement of the oral cavity and maxillofacial region has been increasingly reported. Its heterogeneous clinical presentation and the overlap of its features with various autoimmune, inflammatory, and neoplastic disorders make IgG4-RD a significant diagnostic challenge in Dentistry. Advances in the understanding of the immunopathological mechanisms and histological alterations associated with the disease have contributed to a better comprehension of its pathophysiology and its manifestations within oral tissues. The most frequently reported oral manifestations include salivary gland enlargement, xerostomia, pseudotumoral swellings, palatal involvement, and oral mucosal alterations, which may mimic other conditions such as Sjögren’s syndrome (SS), chronic sialadenitis, and lymphomas. The aim of this narrative review is to summarize the current knowledge on the immunopathological characteristics of IgG4-RD with oral and maxillofacial involvement in order to reduce the challenges associated with its early diagnosis. The discussion of clinical implications and available therapeutic approaches aims to contribute to a better understanding of IgG4-RD, highlighting the importance of interdisciplinary referral and the integration of the dental practitioner in the diagnostic process, in order to ensure a timely and appropriate intervention.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and variable elevations in serum IgG4 levels. Although IgG4-RD is most commonly recognized in organs such as the pancreas, kidneys, and lacrimal glands, involvement of the oral cavity and maxillofacial region has been increasingly reported. Its heterogeneous clinical presentation and the overlap of its features with various autoimmune, inflammatory, and neoplastic disorders make IgG4-RD a significant diagnostic challenge in Dentistry. Advances in the understanding of the immunopathological mechanisms and histological alterations associated with the disease have contributed to a better comprehension of its pathophysiology and its manifestations within oral tissues. The most frequently reported oral manifestations include salivary gland enlargement, xerostomia, pseudotumoral swellings, palatal involvement, and oral mucosal alterations, which may mimic other conditions such as Sjögren’s syndrome (SS), chronic sialadenitis, and lymphomas. The aim of this narrative review is to summarize the current knowledge on the immunopathological characteristics of IgG4-RD with oral and maxillofacial involvement in order to reduce the challenges associated with its early diagnosis. The discussion of clinical implications and available therapeutic approaches aims to contribute to a better understanding of IgG4-RD, highlighting the importance of interdisciplinary referral and the integration of the dental practitioner in the diagnostic process, in order to ensure a timely and appropriate intervention.
Descrição
Dissertação para obtenção do grau de Mestre no Instituto Universitário Egas Moniz
Palavras-chave
Doença relacionada à IgG4 Cavidade oral Diagnóstico diferencial Imunopatologia