Update on the diagnosis and management of Behçet’s disease

Abstract

Behçet’s disease is a multi-organ disorder that is more common in countries around the Silk Road, and manifests as mucosal ulcers and skin lesions, and with ocular involvement. As a systemic disease, it can also involve gastrointestinal organs and the central nervous or cardiovascular systems. Although the etiology of Behçet’s disease is not clearly identified, the pathogenesis of the disease is most commonly hypothesized as a profound inflammatory response triggered by an infectious agent in a genetically susceptible host. As there are no single specific manifestations or specific diagnostic tests, various diagnostic criteria have been proposed around the world, and, among them, the International Study Group criteria have been most commonly used. As the clinical expression of Behçet’s disease is heterogeneous, the treatment should be individualized based on involved organs, severity of the disease, and patient’s background. The choice of therapeutic agents is limited by lack of clinical trials and is based largely on case reports, case series, and several open-label clinical trials. Corticosteroids, colchicine, and traditional immunosuppressive agents, including azathioprine and cyclosporine, have been used for the treatment of Behçet’s disease. Recently, tumor necrosis factor (TNF) inhibitors have become available for several rheumatic diseases, and considerable published data suggest that TNF inhibitors represent an important therapeutic advance for patients with severe and resistant disease, as well as for those with contraindications or intolerance to these treatments.

Vasculo-Behçet disease complicated by conversion disorder diagnosed with ¹⁸F-fluoro-deoxy-glucose positron emission tomography combined with computed tomography (PET/CT)

“…According to a report from Turkey, superficial venous thrombosis is the most common venous complication (53%) and deep vein thrombosis is the second most common (30%) 16) . Although the frequency of arterial involvement in VBD is less than that of ve-nous involvement (1 to 7%), the mortality rate in patients with arterial lesions is higher 21,22) . Aneurysm or occlusion in the aorta or pulmonary artery can be a cause of sudden death.…”

Biological agent in prevention of ocular recurrence in Behçets disease: a case report

“…Evidence suggests that the pathogenesis of BD requires an unusual immune response in patients who are genetically predisposed to the disease following exposure to an exogenous agent, probably infective, supporting the involvement of human leukocyte antigen B51 (HLA-B51) (McNally TW et al, 2017). Infectious agents, such as Streptococcus sanguis, Herpes simplex virus, hepatitis viruses, and parvovirus B19 have been implicated as causes of Behçet's disease (Ryo Rokutanda et al, 2015).…”

“…Corticosteroids, Colchicine, and traditional immunosuppressive agents, including Azathioprine and Cyclosporine, have been used for treating Behçet's disease (Ryo Rokutanda et al, 2015). Recently, considerable published data suggest that TNF inhibitors such as Adalimumab represent a significant therapeutic advance for patients with severe and resistant disease, as well as for those with contraindications or intolerance to these treatments (Ryo Rokutanda et al, 2015).…”

Unusual association of leptomeningitis, pachymeningitis, dural sinus thrombosis, posterior uveitis and pathergy in alady with Behcet’s disease

“…A score >3 is suggestive of Behcet's disease (5) . Pathergy test, which is a classical feature of Behcet's is not seen in all patients and hence is not diagnostic.…”