Behçets Disease as an Autoinflammatory Disorder

Abstract

Autoinflammatory diseases are a group of heritable disorders that are characterized by seemingly unprovoked episodes of inflammation at certain locations and and relative lack of high-titer autoantibodies or antigen-specific T cells. Behçet's disease is an inflammatory disorder of unknown aetiology, and many of its characteristic recurrent manifestations overlap with those of autoinflammatory diseases. Behçet's disease has a complex genetic aetiology, and it is more prevalent in certain geographic regions and/or in particular ethnic groups. Enhanced inflammatory response and over-expression of proinflammatory cytokines are the prominent features of Behçet's disease, and they are compatible with the findings in other autoinflammatory disorders. There are also evidences of antigen-driven immune response in Behçet's disease, but it possibly develops on the background of enhanced innate immune reactivity. Delineation of the similarities of Behçet's disease to other hereditary autoinflammatory diseases may help to clarify its pathogenesis and also to identify the missing links in the shared inflammatory pathways.

CSF/serum matrix metallopeptidase‐9 ratio discriminates neuro Behçet from multiple sclerosis

“…Based on our observations, we hypothesize the occurrence of a distinctive inflammatory environment in CSF of MS and NDB. In NBD CSF, TNF α and IL6 levels were higher in respect to MS ones; overall, the measured levels of CXCL8, IL6, and TNF α confirm the inflammatory response, previously described in NBD and in Behçet disease 20, 21. We know that neutrophils are important in Behçet disease inflammation 22 and the presence of CXCL8 in CSF suggests their involvement in the neuronal form too.…”

Association of Long Noncoding RNAs Polymorphisms With Ankylosing Spondylitis, Vogt-Koyanagi-Harada Disease, and Behcet's Disease

“…BD is an inflammatory disorder with a complex genetic background characterized by uveitis, oral aphthae, skin lesions, and genital ulcers, whereas VKH disease is a systemic autoimmune disease that targets melanocytes not only in the uvea, but also elsewhere in the body, resulting in multiple systemic abnormalities leading to dysacusis, alopecia, poliosis, and vitiligo. 62,63 As mentioned above, rs4937362 was associated with AS in our Chinese Han population but not with the uveitis entities tested. The SNP rs4937362 is located at 11q24.3 in lncRNA RP11-264E20.1 and has been shown to be strongly associated with follicular lymphoma (P ¼ 6.76 3 10 À11 ).…”

Juvenile Behсet's Disease: Clinical Observation

“…При этих заболева-ниях чаще всего поражается кожный покров, серозные оболочки, суставы, ЖКТ, глаза; воспалительный процесс сопровождается повышенным уровнем белков острой фазы воспаления, но относительно низким уровнем ауто-антител или антигенспецифичных Т клеток [49]. В пользу аутовоспалительной природы болезни Бехчета свиде-тельствуют повышение активности нейтрофилов и уровня интерлейкина 1␤ [50], периодические эпизоды ремис-сии и обострения, гиперэкспрессия провоспалительных цитокинов [51]. Более того, имеются сведения, что при болезни Бехчета нередко встречаются некоторые вари-анты гена периодической болезни [52].…”