Curr Opin Immunol. 2025 Jun 27;95:102597. doi: 10.1016/j.coi.2025.102597. Online ahead of print.
ABSTRACT
Although we can at present more effectively manage Behçet syndrome compared to several decades ago, we are not yet quite near a cure. Our main shortcoming is we have yet to decipher its cause(s) and disease mechanism(s). First, there are reasons to consider Behçet syndrome as more than one condition. Among these are (i) distinct phenotypes and different affected organ clusters in disease expression; (ii) marked differences in organ response to any one of several agents used in treatment; and (iii) considerable variation in disease expression between different geographies and ethnicities. Examples include separate clustering of venous and arterial disease, the differing frequency of the pathergy reaction and HLA B51 association among different geographies with its absence in gastrointestinal disease and the good response of many manifestations to anti-tumor necrosis factor (TNF) agents with no response of the pathergy reaction to the same agents. Another issue is that males have considerably more severe disease course than females. Up to now, this has attracted neither enough basic science research nor has it been duly accounted for in the design, analysis, and interpretation of our clinical research, notably as related to randomized trials. Finally, we consider the attempts to lump Behçet syndrome with other conditions to be not helpful to decipher the nature of this condition. With this review, we reemphasize how the lumping of MHC-I-opathy or Behçet spectrum of diseases does not help us much in understanding Behçet better and underline we should be splitters rather than lumpers to this end.
PMID:40580780 | DOI:10.1016/j.coi.2025.102597