title: Immunopathogenesis of ocular Behçet's disease.
abstract: Behçet's disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet's uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.
authors: Department of Ophthalmology, National Medical Center, 245 Euljiro, Jung-gu, Seoul 100-799, Republic of Korea.
authors: Un Chul
authors: UC
authors: Park
authors: Department of Ophthalmology, Seoul Metropolitan Government Seoul National University Boramae Medical Center, No. 395 Shindaebang-2-dong, Dongjak-gu, Seoul 156-707, Republic of Korea ; Department of Ophthalmology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 110-799, Republic of Korea.
authors: Tae Wan
authors: TW
authors: Kim
authors: Department of Ophthalmology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 110-799, Republic of Korea ; Sensory Organs Institute, Medical Research Center, Seoul National University, 103 Daehak-ro, Jongno-gu, Seoul 110-799, Republic of Korea.
authors: Hyeong Gon
authors: HG
authors: Yu
mesh_terms: D000941:Antigens / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D001528:Behcet Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D001688:Biological Products / Q000627:therapeutic use; D018925:Chemokines / Q000378:metabolism; D016207:Cytokines / Q000378:metabolism; D020022:Genetic Predisposition to Disease; D006801:Humans; D007107:Immune System / Q000166:cytology / Q000276:immunology / Q000378:metabolism
chemical_list: D000941:Antigens; D001324:Autoantigens; D001688:Biological Products; D018925:Chemokines; D016207:Cytokines