Behcet’s disease

Abstract

Behcet's disease (BD) is a systemic disorder of recurrent acute inflammation, characterized by major symptoms of oral aphthous ulcers, uveitis, skin lesions and genital ulcers. Involvement of intestines, vessels, and central nervous system (CNS) sometimes leads to a poor prognosis. Patients with BD are known to distribute along the ancient Silk Road. The incidence is relatively higher from eastern Asia to the Mediterranean area as roughly 1-10 patients in 10,000 people, whereas only 1-2 patients in 1,000,000 people in UK and North America. Although etiology of the disease is still unknown, high prevalence of HLA-B51, increased expression of heat shock protein 60 and Th1 dominant immune responses in the patients are considered important in its pathogenesis. Non-infectious neutrophil activation and infection with Streptococcus sanguis and herpes simplex virus would also be associated. Because BD lacks any pathognomonic symptoms and laboratory findings, the diagnosis relies largely upon the criteria proposed by the International Study Group for Behcet's disease in 1990. In Japan, the diagnosis was also made according to the Japanese criteria revised in 1987. Recently, the Behcet's Disease Research Committee of Japan again revised the Japanese criteria in 2003 to avoid overdiagnosis. The new Japanese criteria are introduced in this review. Differential diagnosis excluding Sweet's disease, pemphigus, erythema nodosum and Crohn's disease is important, and positive laboratory data for pathergy test, prick test for dead Streptococci and HLA-B51 are emphasized to make appropriate diagnosis in these criteria. Pathological findings of the disease-affected site such as erythematous nodosum is also stressed. Treatment for the disease has been chosen according to the clinical symptoms. Non-steroidal anti-inflammatory drugs, immunosuppressants, corticosteroids and colchicine are basically introduced. Recently, effects of interferon-alpha/beta, anti-tumor necrosis factor antibody and thalidomide are encouraging, specifically in treatment for the cases with poor prognosis including eye, intestine, vessel and CNS involvement. Low dose weekly administration of methotraxate looks effective for the cases with CNS involvement. Further studies for elucidation of the etiology, improvement of the diagnostic criteria and development of new therapy are needed to conquer the disease.

Keratic precipitate morphology in uveitic syndromes including Behçet's disease as evaluated with in vivo confocal microscopy

“…Behçet's disease is a multisystem disorder with unknown aetiology characterized histopathologically by occlusive vasculitis. 13 Although infectious agents, 14 autoimmune mechanisms, 15 and genetic factors 14 have been implicated in the immunopathogenesis of Behçet's disease, the exact mechanism(s) remains obscure. The anterior and/or the posterior segment may be involved in Behçet's disease in the form of non-granulomatous intraocular inflammation.…”

Ocular involvement is associated with HLA-B51 in Adamantiades–Behçet's disease

“…19 Among different genetic markers, the class I HLA-B5 and -B51 gene on chromosome 6p21 is in association with ABD. 20,21 The relative risk of ABD in HLA-B51-positive patients is 13.3 for Turkey, 6.7 for Japan, and 1.3 for the United States of America. 22 HLA-B51 and its suballeles, B5101 and 5121, are important for antigen synthesis of CD8 þ cytotoxic suppressor T cells.…”

Characterization of the Divergent Wound-Healing Responses Occurring in the Pathergy Reaction and Normal Healthy Volunteers

“…It typically manifests as recurrent oral and genital ulcerations and uveitis, variably accompanied by symptoms affecting skin, the large vessels, gastrointestinal system, and CNS or other organs (1). The precise mechanisms of tissue destruction in BD have not been fully elucidated.…”