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Behcet's Disease
Published in Charles Theisler, Adjuvant Medical Care, 2023
Behcet’s disease is a multiorgan vasculopathic inflammatory condition characterized by a triad of recurrent episodes of oral aphthous (mouth) ulcers and genital sores, as well as ocular inflammation (anterior and posterior uveitis). Repeated attacks of uveitis can cause blindness.1 Additional symptoms can include arthritis, colitis, and CNS involvement. The diagnosis is based on mouth and groin sores recurring at least three times in 12 months. Behcet’s disease usually develops in young adults aged 20–30 years, but patients of all ages, gender, and races may be affected. Behcet’s disease is not contagious. Treatment of Behcet’s disease is symptomatic and is aimed at reducing symptoms and preventing complications.2,3 Corticosteroids and colchicine are frequently used in medical treatment.
Unexplained Fever Associated With Hypersensitivity and Auto-Immune Diseases
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Behcet’s disease can be defined as the association with aphtae (ulcers) of the mouth and the genitalia, of multisystemic disease: ocular symptoms (relapsing iritis), vascular thrombosis (thrombophlebitis) cutaneous, articular, nervous system lesions . . .
High-Dose Chemotherapy with Haematopoietic Stem Cell Transplantation in Primary Systemic Vasculitis, Behcet’s Disease and Sjogren’s Syndrome
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Christoph Fiehn, Manfred Hensel
In Behcet’s disease, in particular, arterial vascular involvement can be life threatening.’21-23 About 4% of the patients with Behcet’s disease have arterial involvement, with vasculitis of the pulmonary artery which results in pulmonary arterial aneurysms being the most frequent manifestation.2,4 In an overview of 24 cases with this type of vasculitis, 50% of the patients died after a mean of 9.5 month.4
Quantitative Analysis of Optical Coherence Tomography Angiography Features in Patients with Nonocular Behcet’s Disease
Published in Current Eye Research, 2019
Yasin Sakir Goker, Sedat Yılmaz, Hasan Kızıltoprak, Kemal Tekin, Gokhan Demir
Behcet Disease is a chronic occlusive vasculitic syndrome that can affect small and large blood vessels including retinal vessels, the pulmonary artery, and the aorta.21 There is also a high prevalence of venous deficiency in patients with asymptomatic BD.22 FA is the gold-standard imaging modality that allows for evaluation of the non-perfusion area and FAZ in patients with BD. Khairallah et al. reported that OCTA is more sensitive than FA in evaluating macular perfusion in Behcet uveitis.16 Additionally, OCTA allows physicians to quantitatively assess foveal density-300 (FD-300) (%) (vessel density in 300 microns around the FAZ) and vessel densities of the foveal, parafoveal, and perifoveal regions.18 FD-300 is a crucial parameter in the OCTA FAZ assessment tool that gives information about macular perfusion.18 In our study, we found that FD-300 in full retinal vasculature was significantly lower in the BD group than in the control group (p = 0.045). Also, vessel densities in the foveal region of both SCP and DCP were significantly lower in the BD group than in the control group (p = 0.039 and p = 0.010, respectively). We hypothesize that lower capillary vessel densities, particularly in the foveal region, could be considered a subclinical form of ocular involvement in nonocular BD.
Long-Term Efficacy and Safety of Interferon Alpha-2a in the Treatment of Chinese Patients with Behçet’s Uveitis Not Responding to Conventional Therapy
Published in Ocular Immunology and Inflammation, 2019
Peizeng Yang, Guo Huang, Liping Du, Zi Ye, Ke Hu, Chaokui Wang, Jian Qi, Liang Liang, Lili Wu, Qingfeng Cao, Aize Kijlstra
Recently a number of prospective and retrospective studies have addressed the safety and curative efficacy of IFNα-2a in the treatment of patients with refractory BD. A variety of therapeutic regimens have been developed using different strategies and doses of IFNα-2a in the treatment of BD.10,11,17–23 IFNα-2a treatment combined with a low dose of corticosteroids without immunosuppressants was common in previous studies. A study by Deuter et al. has recommended IFN monotherapy or a combination of IFN and corticosteroids at a dose of less than 10 mg/day in an attempt to avoid blocking of NFkB by corticosteroids and competition with the IFN effect.9 However, Bodaghi et al. have advised the use of intravenous pulses of corticosteroids for 3 days followed by a high oral dose during the first month of IFN therapy.23 In general, both studies show a therapeutic effect in Behcet’s disease. In the present study, we initially used IFN in combination with a low dose of corticosteroids (20 mg/day) with a gradual tapering of this drug. Our results also showed a positive effect on BD patients who did not respond to conventional therapy. The combination of IFN therapy with corticosteroids in the treatment of BD is still a matter for debate and more studies are needed to clarify this issue.
Epidemiology of Behcet’s Disease in Taiwan: A Population-Based Study
Published in Ophthalmic Epidemiology, 2018
Yu-Hao Lin, Tzu-Yang Tai, Cheng-Yun Pu, De-Kuang Hwang, Yu-Mei Chung, Yiing-Jeng Chou
Behcet’s disease is an idiopathic inflammatory disease that can cause chronic and recurrent vasculitis in multiple organs.1 Behcet’s disease is usually diagnosed based on clinical features, including uveitis, oral ulcers, genital ulcers, skin lesions, neurological signs, vascular manifestations, and positive responses to pathergy tests.2 Incidence of Behcet’s disease is distributed worldwide, with the highest prevalence in countries along the ancient Silk Route bridging Asia, the Middle East, and the Mediterranean region.3–5 Prevalences of Behcet’s disease per 100,000 people have been reported as 20–421 in Turkey, 80 in Iran, 20 in Saudi Arabia, 17 in Iraq, and 13.5 in Japan. By contrast, Behcet’s disease is less common in Western Europe, the United States, and the rest of the world. Prevalences per 100,000 people have been estimated as 0.64 in the United Kingdom, 0.7–2.3 in Germany, 5.2 in the United States, and 7.1 in France.6–14 However, information regarding incidence of Behcet’s disease in the Chinese population is scarce.