Pathophysiology of the Behçet's Disease

Abstract

Behçet's disease (BD) is a multisystemic disease of unknown etiology characterized by chronic relapsing oral-genital ulcers and uveitis. Multiple systemic associations including articular, gastrointestinal, cardiopulmonary, neurologic, and vascular involvement are also observed in BD. Although the etiopathogenesis of the disease remains unknown, increased neutrophil functions such as chemotaxis, phagocytosis, and excessive production of reactive oxygen species (ROS), including superoxide anion, which may be responsible for oxidative tissue damage seen in BD, and also immunological alterations, T lymphocyte abnormalities in both subpopulation and function have been considered to be correlated with the etiopathogenesis of BD. There is some clinical evidence suggesting that emotional stress and hormonal alterations can influence the course and disease activity of BD.

Neutrophil–Lymphocyte Ratio and Carotid–Intima Media Thickness in Patients With Behçet Disease Without Cardiovascular Involvement

“…Cytokines, which were predominantly known in various immune mechanisms, were synthesized by various cell types as important mediators of immune inflammatory reactions. 16 Proinflammatory cytokine and mediators could be involved in prognosis of BD. 16 Endothelial dysfunction, another characteristic of BD, can be observed even in patients without clinical vascular involvement.…”

A Case of Behçet’s Disease and Systemic Sclerosis Developing After an Earthquake Disaster

“…Consequently, SSc has been considered to be a Th2 cell -dominant disease. In contrast, BD is considered to be a Th1 -type autoimmune disease because Th1 -cell predominant cytokines, such as TNF -α, IL -2, IL -8, and IFN -γ, are increased in patients with BD 2,3) . Recently, Th17 cells, which produce the typical cytokine IL -17, have also been implicated in the pathologies of SSc and BD.…”

“…However, increased functions of neutrophils such as chemotaxis, phagocytosis, and the overproduction of superoxide may cause tissue damage in patients with BD. Furthermore, clinical evidence suggests that hormonal alterations and emotional stress affect the clinical course and disease activity of BD 2,3) . SSc is usually considered a Th2 -dominant disease because fibrogenesis can be induced when Th2 cytokines (IL -4, IL -13) predominate over Th1 cytokines.…”

Serum anti-lysozyme is associated with disease activity of Behçet's disease

“…Although the immunologic mechanism in the pathogenesis of BD still remains, αβT cells of γδT cells initiate the pathologic reactions by hypersensitivity to multiple antigens, and activate monocytes, through CD40–CD154 interaction, leading to T cell‐activation. Activated monocytes and T cells, in turn, produce cytokines, including tumor necrosis factor (TNF)‐α, interferon (IFN)‐γ, interleukin (IL)‐1, IL‐6, IL‐8, IL‐17 and granulocyte‐macrophage colony‐stimulating factor (GM‐CSF), which may trigger the activation of neutrophils and drive them to provoke tissue damage through chemotaxis, reactive oxygen species production and phagocytosis along with site‐infiltration, accompanied by a complex interplay with Th1‐phenotype cell responses during active BD …”