Etiopathogenesis of Behçet's disease with emphasison the role of immunological aberrations

Abstract

Behçet's disease (BD) is a chronic multisystemic inflammatory disorder of unknown origin consisting of oral aphthous ulcers, ocular symptoms, skin lesions, and genital ulcerations. It has many features in common with systemic vasculitides and is more prevalent in countries along the ancient Silk route. Immune-mediated mechanisms play a major role in the pathogenesis of the disease, and inflammatory mediators are also involved. BD is not considered to be an autoimmune disorder, and the character of the disease needs to be clarified. Immunological aberrations in BD have been extensively studied by many investigators; genetic factors have been related to disease susceptibility, but their exact role in the development of disease is uncertain. Environmental factors such as infectious agents have also been implicated in the etiology of BD. However, the etiopathogenesis of the disease remains to be elucidated. Factors involved in the immunopathogenesis of BD with emphasis on the role of immunological aberrations are analyzed in this review.

Gene expression profiling and association studies implicate the neuregulin signaling pathway in Behçet's disease susceptibility

“…To the best of our knowledge, we performed the first genome-wide study comparing the expression profiles of BD patients and controls. PBMCs were considered a relevant tissue for expression profiling since BD is a generalized vasculitis characterized by a profound inflammatory and immune dysregulation [1]. Furthermore, given that BD is a complex disease with multiple genes and environmental factors implicated in its etiology, we did not expect to find genes with major expression differences.…”

“…Its most common clinical manifestations are recurrent orogenital ulcerations and inflammatory eye lesions. Although its etiopathogenesis remains to be elucidated, genetic (e.g., HLA-B51) and environmental factors as well as immune-mediated mechanisms have been pointed out as important factors [1].…”

Microbial Infections as an Etiology of Behcet’s Disease

“…They have highly conserved amino acids sequence. There is sequence homology between HSP65 in bacteria, and human mitochondrial HSP60 (Kapsimali et al, 2010).…”

Immunopathogenesis Based Adamantiades- Behcet Disease Vasculitis Treatment

“…Besides, the option of exposure to chemicals was not supported for long. However, although immunological abnormalities concerning innate and adaptive immunity in ABD have been extensively studied, there is no adequate proof that ABD is either a typical autoimmune disease-it does not fulfill substantial criteria-or an auto-inflammatory disease-it is a very weak hypothesis at the moment Kapsimali et al, 2010;Mumcu et al, 2007;Zouboulis & May, 2003). ABD is a systemic vasculitis which may affect arteries and veins of all sizes in any organ and underlies disease manifestations.…”