Jornal Vascular Brasileiro
https://jvascbras.org/article/doi/10.1590/1677-5449.200170
Jornal Vascular Brasileiro
Review Article

Acometimento vascular na doença de Behçet: o processo imunopatológico

Vascular involvement in Behçet’s disease: the immunopathological process

Raquelle Machado de Vargas; Maria Luiza Nunes da Cruz; Maria Paula Hashimoto Giarllarielli; Beatriz Mota Sano; Geovana Idelfoncio da Silva; Karina Furlani Zoccal; Cristiane Tefé-Silva

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Resumo

Resumo: A doença de Behçet constitui uma forma rara de vasculite sistêmica, que acomete de pequenos a grandes vasos. É caracterizada por manifestações mucocutâneas, pulmonares, cardiovasculares, gastrointestinais e neurológicas. Sua apresentação clínica é bastante ampla, variando de casos mais brandos a casos graves, com acometimento multissistêmico, caracteristicamente com exacerbações e remissões. Suas causas ainda são desconhecidas; entretanto, há evidências genéticas, ambientais e imunológicas, como a associação com o alelo HLA-B51. Todas essas, em conjunto, apontam para um processo imunopatológico anormal, com ativação de células da imunidade inata e adaptativa, como as células natural killer, neutrófilos e células T, que geram padrões de respostas e citocinas específicos capazes de gerar mediadores que podem lesionar e inflamar o sistema vascular, resultando em oclusões venosas, arteriais e/ou formação de aneurismas.

Palavras-chave

síndrome de Behçet, antígeno HLA-B51, vasculite sistêmica

Abstract

Behçet’s disease is a rare form of systemic vasculitis that affects small to large vessels. It is characterized by mucocutaneous, pulmonary, cardiovascular, gastrointestinal, and neurological manifestations. Its clinical presentation is quite wide, ranging from milder cases to severe cases, with multisystemic involvement, characteristically with exacerbations and remissions. Its etiopathogenesis is still unclear, although there is evidence of genetic, environmental, and immunological factors, such as the association with the HLA-B51 allele. In conjunction, all of these point to an abnormal immunopathological process, with activation of cells of innate and adaptive immunity, such as NK cells, neutrophils, and T cells, which generate specific response patterns and cytokines capable of generating mediators that can damage and inflame blood vessels, resulting in venous and arterial occlusions and/or aneurysm formation.

Keywords

Behçet syndrome; HLA-B51 antigen; systemic vasculitis.

References

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Submitted date:
09/08/2020

Accepted date:
02/25/2021

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