Nickan Research Institute Immunopathologia Persa 2423-8015 9 1 2023 01 01 Behcet’s disease; a narrative review with a focus on autoimmunity processes in involved organs e29262 e29262 10.34172/ipp.2022.29262 EN Zeynab Ebrahimi Fard https://orcid.org/0000-0003-2205-2859 Javad Balasi https://orcid.org/0000-0002-1588-5169 Atiyeh Kimiaefar https://orcid.org/0000-0001-6236-4799 Kimia Jazi https://orcid.org/0000-0001-5198-5709 Zahra Hajrezaei https://orcid.org/0000-0003-0507-4335 Jafar Karami https://orcid.org/0000-0002-2554-1669 Seyyed Mohsen Banitaba Maryam Masoumi https://orcid.org/0000-0003-2635-2656 Journal Article 10.34172/ipp.2022.29262 2021 08 11 2021 12 13 Behcet’s disease (BD) is an autoimmune and inflammatory disease that is mainly characterized by involvement of the gastrointestinal tract, cardiovascular system, genital tract and joints. The disease is more common in the Middle Eastern and Far Eastern countries and the frequency of recurrences and attacks varies among populations. Although the pathogenesis is not fully understood, there is evidence to suggest that immunodeficiency and specific patterns of production of immune regulatory mediators are important. Impaired autoimmune and inflammatory cells, target endothelial cells and prompt vasculitis in the involved organs. Elucidating the immunological mechanism of BD can facilitate the development of new therapies and therapeutic immunoregulatory interventions to prevent possible complications. New therapies, including manipulation of immune-regulating mediators such as interferon-alpha antagonists, have shown promising results in patients with BD. This review aims to consider the role of auto-inflammatory and autoimmune processes and related factors which give rise to the different manifestations of the disease in each involved organ.