Behçet's disease physiopathology: a contemporary review

Mohamad J Zeidan, David Saadoun, Marlene Garrido, David Klatzmann, Adrien Six, Patrice Cacoub, Mohamad J Zeidan, David Saadoun, Marlene Garrido, David Klatzmann, Adrien Six, Patrice Cacoub

Abstract

Behçet's disease, also known as the Silk Road Disease, is a rare systemic vasculitis disorder of unknown etiology. Recurrent attacks of acute inflammation characterize Behçet's disease. Frequent oral aphthous ulcers, genital ulcers, skin lesions and ocular lesions are the most common manifestations. Inflammation is typically self-limiting in time and relapsing episodes of clinical manifestations represent a hallmark of Behçet's disease. Other less frequent yet severe manifestations that have a major prognostic impact involve the eyes, the central nervous system, the main large vessels and the gastrointestinal tract. Behçet's disease has a heterogeneous onset and is associated with significant morbidity and premature mortality. This study presents a current immunological review of the disease and provides a synopsis of clinical aspects and treatment options.

Keywords: Autoimmunity; Behçet disease; Inflammation; Physiopathology.

Conflict of interest statement

Compliance with ethical standards Funding None. Conflict of interest All authors declare that they have no conflict of interest. Ethical approval This article does not contain any studies with human participants or animals performed by any of the authors.

Figures

Fig. 1
Fig. 1
Summary of pathogenesis of Behçet disease. It is presumed that environmental factors trigger CD4+ T cells activation in genetically susceptible individuals, leading to the secretion of cytokines that stimulate other inflammation-inducing immune cells resulting in an uncontrolled autoimmune cascade in vascular tissue. SNP single nucleotide polymorphism, IL interleukin, HLA human Leukocyte Antigen, HSP heat shock proteins, INFγ interferon gamma, TNF-α tumor necrosis factor alpha, CD mature Th cells expressing a surface protein identified by a number, NKT natural killer T cells, Th T helper cells, ϒσ T cells T lymphocytes

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