Vascular involvement in Behçet's disease: the immunopathological process

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.

Figure 1. Flow diagram illustrating selection of articles.

Figure 2. Diagram illustrating the immunopathological mechanism of Behçet’s disease. The HLA-B51 allele and infections mediate pathogenesis of Behçet’s disease. These triggers inappropriately activate an immune system capable of producing cytokines and, consequently, reactive cells, injuring vessels as a consequence. IL = interleukin; IFN = interferon; TNF = tumor necrosis factor.

Figura 1. Fluxograma de seleção de artigos.

Figura 2. Diagrama do mecanismo imunopatológico da doença de Behçet - o alelo HLA-B51 associado às infecções medeiam a patogenia da doença de Behçet. Tais gatilhos ativam um sistema imunológico inadequadamente capazes de produzir citocinas e, consequentemente, células reativas, lesionando, por fim, os vasos. IL = interleucina; IFN = interferon; TNF = fator de necrose tumoral.