Behcet's Disease, and the Role of TNF-α and TNF-α Blockers

In this both narrative and systematic review, we explore the role of TNF-alpha in the immunopathogenesis of Behcet's disease (BD) and the effect of treatment with TNF-alpha blockers. BD is an auto-inflammatory disease, characterized by recurrent painful oral ulcerations. The pathogenesis of BD is not yet elucidated; it is assumed that TNF-alpha may play a key role. In the narrative review, we report an increased production of TNF-alpha, which may be stimulated via TLR-signaling, or triggered by increased levels of IL-1 beta and IFN-gamma. The abundance of TNF-alpha is found in both serum and in sites of inflammation. This increased presence of TNF-alpha stimulates T-cell development toward pro-inflammatory subsets, such as Th17 and Th22 cells. Treatment directed against the surplus of TNF-alpha is investigated in the systematic review, performed according to the PRISMA guideline. We searched the Pubmed and Cochrane database, including comparative studies only. After including 11 studies, we report a beneficial effect of treatment with TNF-alpha blockers on the various manifestations of BD. In conclusion, the pivotal role of TNF-alpha in the immunopathogenesis of BD is reflected in both the evidence of their pro-inflammatory effects in BD and in the evidence of the positive effect of treatment on the course of disease in BD.