| Behc¸et’s disease (BD) is a chronic autoimmune condition primarily prevalent in populations along the
Mediterranean Sea. The exact etiology of BD has not been fully explained yet, but the disease occurrence
is associated with a genetic factor, human leukocyte antigen (HLA)-B51 antigen. Among the various immu-
nodysfunctions that are found in BD, patients are increased neutrophil motility and superoxide produc-
tion, as well as elevated production of tumor necrosis factor (TNF)-a and decreased production of
interleukin (IL)-10. Elevated levels of inflammatory cytokines like IL-1 and IL-17 in BD have been found
associated with aberrant expression of microRNA. Gene polymorphisms in BD patients have been
observed in molecules involved in responses to pathogens that can ultimately modulate the host anti-
microbial response. Moreover, several single nucleotide polymorphisms (SNPs) have been reported in
genes encoding chemokines and adhesion molecules; many of these changes manifest as increases in vas-
cular inflammation and vascular damage. Lastly, genetic and epigenetic changes have been suggested as
involved in the pathogenesis of BD. Modifications in DNA methylation have been found in BD patient
monocytes and lymphocytes, leading to adverse function of these cells. This review presents a compre-
hensive compilation of the literature with regard to the immunodysfunction underlying BD, as well as of
the genetics, newly described clinical specifications and novel treatment strategies using immunomodu-
lants based on the current understanding of BD. |