Background: Behcet's disease (BD) is a chronic, inflammatory multisystemic condition of unknown etiology. Although the cause of BD is not clear, it is believed to be the result of an autoimmune process triggered by an infectious or environmental agent (possibly local to a geographic region) in a genetically predisposed individual. Objective: To detail current knowledge of the role of microorganisms in the pathogenesis of BD and review the infectious etiology of this disease. Methods: The review

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... based on publication in SCOPUS, Science direct, and PubMed. Results: A microbial infection has been implicated in the development of the disease to explain the strong inflammatory reactions observed, the activation of monocytes and macrophages, and the induction of proinflammatory cytokines and chemokines detected. Common factors linking some of the possible pathogenetic agents are extrinsically induced tissue stress or heat shock proteins, which react with host tissues and elicit significant T-helper type 1 cell responses. Conclusion: Based on collected data, we conclude that the microorganisms discussed seem to participate and, at least in part, act as triggers during the course of BD. By clarifying the microbial associations of BD and finding its etiology, particularly the causative antigens leading to BD, it would be easier to suggest more effective treatment and preventive strategies for this disease.