Behcet Syndrome (Behcet's Syndrome)

Rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region and uveitis with hypopyon. The neuro-ocular form may cause blindness and death. SYNOVITIS; THROMBOPHLEBITIS; gastrointestinal ulcerations; RETINAL VASCULITIS; and OPTIC ATROPHY may occur as well.

Also Known As:

Behcet's Syndrome; Behcet Disease; Adamantiades-Behcet Disease; Behcet Triple Symptom Complex; Behçet Disease; Old Silk Route Disease; Triple Symptom Complex; Adamantiades Behcet Disease; Adamantiades-Behcet Diseases; Behçet Diseases; Symptom Complex, Triple; Triple Symptom Complices; Behcet's Disease; Triple-Symptom Complex

Networked: 1431 relevant articles (104 outcomes, 172 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Uveitis
2.	Aneurysm (Aneurysms)
3.	Psoriasis (Pustulosis Palmaris et Plantaris)
4.	Retinal Vasculitis
5.	Atopic Dermatitis (Atopic Eczema)

Experts

1.	Yang, Peizeng: 41 articles (04/2022 - 04/2002)
2.	Kijlstra, Aize: 31 articles (01/2021 - 08/2008)
3.	Shahram, Farhad: 16 articles (08/2018 - 08/2009)
4.	Hou, Shengping: 15 articles (11/2016 - 07/2010)
5.	Khabbazi, Alireza: 14 articles (08/2022 - 05/2014)
6.	Davatchi, Fereydoun: 13 articles (02/2022 - 08/2009)
7.	Hamzaoui, Kamel: 12 articles (01/2022 - 01/2004)
8.	Direskeneli, Haner: 11 articles (01/2019 - 02/2005)
9.	Cao, Qingfeng: 9 articles (04/2022 - 01/2016)
10.	Lee, Eun-So: 9 articles (04/2017 - 05/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Behcet Syndrome:

1.	Infliximab (Remicade)FDA Link 03/01/2017 - "Mucosal Healing at 14 Weeks Predicts better Outcome in Low-dose Infliximab Treatment for Chinese Patients with Active Intestinal Behcet's Disease." 07/01/2020 - "Our case is the youngest adult reported so far and is an unusual presentation of neuro-Behcet's disease, as the patient was already in remission and on infliximab infusion. " 02/01/2017 - "To compare the efficacy of infliximab (IFX) on refractory uveoretinitis between Behçet disease (BD) patients with short duration (≤18 months, n = 6) versus long duration (>18 months, n = 7) of their ocular disease. " 12/01/2020 - "This study aims to investigate the efficacy and reliability of infliximab treatment in Behcet's disease with vascular involvement. " 01/01/2014 - "Experimental studies have shown the supreme role of infliximab in the management of Behcet's disease. "
2.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 12/01/2018 - "Efficacy of anti-TNF alpha in severe and refractory major vessel involvement of Behcet's disease: A multicenter observational study of 18 patients." 12/01/2018 - "This study designed to analyze the miR-146a and miR-155 expression in patients with Behcet's disease (BD) and investigated their association with the expression of tumor necrosis factor-alpha (TNF-α) and cytotoxic T lymphocyte associated antigen-4 (CTLA-4) genes. " 01/01/2013 - "Studies investigating the association between the tumor necrosis factor (TNF) gene polymorphisms and Behcet's disease (BD) report conflicting results. " 03/01/2007 - "These results suggest that the variability of the TNF-alpha -1031T/C polymorphism can be associated with the susceptibility to Behcet's disease in our study group. " 04/01/2022 - "This is a systematic review and meta-analysis evaluating the efficacy and safety of anti-tumor necrosis factor (TNF) agents in treating patients with intestinal Behcet's disease. "
3.	Thalidomide (Thalomid)FDA Link 03/01/2008 - "Thalidomide is shown to be an effective treatment for mucocutaneous symptoms of Behcet's disease (BD). " 12/01/2021 - "Here, we present a 59-year-old man who had recurrence of oral and genital aphthosis for 3 years and was diagnosed with Behcet's disease (BD), which was cured by thalidomide. " 03/01/2012 - "Thalidomide has reported efficacy in treating refractory idiopathic aphthous ulceration and aphthous ulceration in Behçet disease (BD). " 04/25/2011 - "SK94 and SK126 were administered to the Behcet's disease-like mice for five consecutive days and SK94 improved in five out of six mice (83%), while it only improved in one out of nine mice (11%) in the pH 1.2 saline (artificial gastric juice) group (P<0.005), four out of ten mice (40%) in the thalidomide group (P<0.05), and six out of seven (86%) in the SK126 group (P<0.005). " 06/01/2010 - "Forty-seven patients were treated with thalidomide for recurrent aphthosis (41 patients) or Behçet disease (six patients). "
4.	SteroidsIBA 02/01/2021 - "A 42-year-old man with a history of suspected of Behcet's disease underwent oral steroid treatment. " 09/01/2020 - "Because she didin't carry the criteria for Behcet's disease, she was diagnosed as Hughes-Stovin Syndrome (HSS) and steroid treatment was started. " 10/18/2016 - "They initially presented with symptoms and signs resembling Behcet's disease and were treated with systemic steroids with suboptimal responses. " 09/01/2008 - "These various manifestations of Behçet disease respond to steroid treatment; however, one of the characteristics of Behçet disease is the high rate of complications and recurrence after surgery. " 11/01/2023 - "Given his constellation of symptoms and improvement with steroids during surgical procedures, he was ultimately diagnosed with a variant of Behcet's disease known as Hughes-Stovin syndrome. "
5.	Adalimumab (Humira)FDA Link 06/01/2010 - "Adalimumab appears to be effective in Behçet disease. " 04/01/2016 - "Long-Term Efficacy of Adalimumab in Patients With Intestinal Behcet's Disease: Eight Consecutive Cases." 01/01/2015 - "Efficacy of Adalimumab in a Girl with Refractory Intestinal Behcet's Disease." 01/01/2023 - "The short- and long-term effects of adalimumab (ADA) on Korean patients with intestinal Behcet's disease (BD) for remain unclear. " 04/01/2016 - "The long-term efficacy and safety of adalimumab (ADA) for the treatment of intestinal Behcet's disease (BD) in the clinical setting have not been evaluated previously. "
6.	Immunosuppressive Agents (Immunosuppressants)IBA 01/01/2023 - "To report the clinical profile of Behcet's disease and its management with immunosuppressants and biologics in a cohort of 25 patients from a tertiary eye care center in South India. " 10/01/2021 - "Similar to other vasculitic disorders, Behcet's disease is primarily treated with immunosuppressants. " 09/01/2021 - "Before the fourth operation, the patient was diagnosed with Behcet's disease and given immunosuppressant. " 01/01/2016 - "He was diagnosed as having Behcet's disease (BD) and subsequently responded to immunosuppressants and anticoagulation. " 11/01/2015 - "Left ventricular intracardiac thrombus in a patient with Behçet disease successfully treated with immunosuppressive agents without anticoagulation: a case report and review of the literature."
7.	Colchicine FDA LinkGeneric 09/01/2023 - "Corrigendum to: "The Role of Colchicine in Different Clinical Phenotypes of Behcet's Disease" [Clin Therapeut 45(2) (2023) 162-176]." 04/07/2023 - "Exploring the pharmacological and adverse reaction mechanism of a drug by network pharmacology strategy: Using colchicine to treat Behcet syndrome as an example." 01/01/2023 - "The Role of Colchicine in Different Clinical Phenotypes of Behcet Disease." 01/01/2015 - "Patients with Behcet's Disease were evaluated for mean platelets volume before colchicine therapy (Group 1) and after 6-month from beginning of colchicine treatment (Group 2). " 01/01/2015 - "The Effect of Colchicine on Mean Platelet Volume in Behcet's Disease."
8.	Cyclosporine (Ciclosporin)FDA LinkGeneric 11/01/2010 - "Relationship between cyclosporine and neuro-Behcet's disease are discussed." 02/01/2005 - "Behçet disease presenting with neurological complications immediately after conversion from conventional cyclosporin A to microemulsion formulation." 04/01/2003 - "Cyclosporine was suggested recently by Japanese workers to provoke central nervous systems symptoms in Behcet`s disease. " 03/01/1999 - "Thus, neurologic complications appear to represent a major side effect of cyclosporine in the treatment of patients with Behçet disease." 03/01/1999 - "Cyclosporine in the treatment of Behçet disease appears to cause neurotoxicity or to accelerate the development of CNS symptoms. "
9.	Adrenal Cortex Hormones (Corticosteroids)IBA 01/01/2021 - "To study the ability of pattern reversal visual evoked potentials (VEPs) to detect subclinical visual pathway pathologic assessment in Behcet disease (BD) and correlate VEP results in BD with disease activity and response to corticosteroid treatment. " 01/01/2021 - "Consistency of Visual Evoked Potential in Extraocular Manifestations of Behcet Disease and Impact of Corticosteroid Treatment." 12/01/2019 - "Auto-immunity profile evaluation during different clinical manifestations of Behçet disease in Algerian patients: effect of corticosteroid treatment." 06/01/2009 - "She suffered from Entero-Behçet disease at the age of 52 and was treated with corticosteroids for 7 years. " 01/01/2007 - "Interrelated modulation of endothelial function in Behcet's disease by clinical activity and corticosteroid treatment."
10.	Interferon alpha-2 (Roferon-A)FDA Link 05/01/1998 - "To evaluate the therapeutic efficacy and safety of systemic recombinant interferon alpha-2a (IFN-alpha) in patients with Behcet's disease (BD) and to determine the incidence of episodes in complete responders during the one-year pretreatment period and follow-up. " 04/01/2002 - "To determine the therapeutic efficacy of interferon alfa-2a in the treatment of Behçet disease. " 04/01/2002 - "Interferon alfa-2a is an effective alternative treatment for Behçet disease, particularly for the management of the mucocutaneous lesions of the disease." 04/01/2002 - "Interferon alfa-2a in the treatment of Behçet disease: a randomized placebo-controlled and double-blind study." 01/01/2019 - "Effect of Interferon alfa-2a Treatment on Adaptive and Innate Immune Systems in Patients With Behçet Disease Uveitis."

Therapies and Procedures

1.	Therapeutics 02/01/2012 - "The aim of this study was to analyze the clinical features, disease activity and therapy of the patients with ophthalmologic manifestation of Behcet's disease. " 01/01/2000 - "Studies were eligible if they fulfilled all of the four following criteria: 1. Randomized controlled trials, single or double-blind; 2. Participants were patients with Behcet's Syndrome as defined by the International Study Group, 1990 (Int Study Group, 1990); 3. Interventions included any pharmacological therapy compared to placebo or some other pharmacological intervention for the treatment of Behcet's syndrome. " 03/19/2020 - "The treatment of intestinal Behcet's disease (BD) is challenging, and one-third of patients require surgery due to failure of conventional therapies. " 01/01/2018 - "Gene Expression Profiling in Behcet's Disease Indicates an Autoimmune Component in the Pathogenesis of the Disease and Opens New Avenues for Targeted Therapy." 09/01/2016 - "Eight hundred fifty-two rheumatologists and 934 ophthalmologists were surveyed via e-mail regarding their choice of therapy for a hypothetical patient with ocular Behçet disease. "
2.	Stents 01/01/2021 - "Stent graft for PAA in patients with Behçet disease had a high early patency rate and minimal morbidity. " 12/01/2017 - "We have implemented a bifurcated stent graft for the patient with a Behcet's disease. " 05/10/2004 - "[Behçet disease with exacerbation in summer and late coronary stent occlusion]." 04/01/2004 - "A 66-year-old woman with a 30-year history of Behcet's disease was referred for coronary surgery because of restenosed stent and crescendo angina. " 09/01/2001 - "The findings in this initial experience suggest that stent-graft insertion may be a safe and effective alternative to surgical treatment of aortic and arterial aneurysms in patients with Behçet disease."
3.	Drug Therapy (Chemotherapy) 04/01/2021 - "This review aimed to investigate the efficacy of integrative medicine (herbal medicine combined with drug therapy) in the treatment of Behcet's disease (BD). " 04/01/2021 - "Integrative Medicine (Herbal Medicine Combined with Drug Therapy) for Behcet's Disease: A Systematic Review and Meta-Analysis of Randomized Controlled Trials." 01/01/2000 - "Pharmacotherapy for Behcet's syndrome." 01/01/1970 - "[Drug therapy in Behcet's disease]." 01/01/2007 - "Two young male patients with severe progressive Behcet's disease with neurological involvement (N-BD) were treated by high-dose immunosuppressive chemotherapy (HIC) followed by autologous CD34+ selected peripheral blood stem cell transplantation (APBSCT). "
4.	Injections 01/01/2006 - "Nine eyes of five patients with Behçet disease with sight-threatening uveitis that was resistant to conventional therapy received repeated IVTA injections. " 01/01/2019 - "In clinical practice, patients with Behcet's disease with retinal vasculitis who received MSC injections experienced a decrease in retinal vasculitis but no improvement in vision acuity. Nonetheless, there is no evidence that MSCs are carcinogenic, and they even reduce the size of tumors in vitro. " 01/01/2006 - "Repeated intravitreal triamcinolone injections in Behçet disease that is resistant to conventional therapy: one-year results." 08/01/2021 - "To study the safety of extended monthly intravitreal infliximab injections in patients with active posterior uveitis in Behcet's disease. " 08/01/2021 - "This is a prospective, interventional, noncomparative, open-label, pilot study of 9 monthly intravitreal infliximab injections (1 mg/0.05 mL) for 22 eyes of 16 patients with active posterior uveitis in Behcet's disease. "
5.	Off-Label Use 09/12/2023 - "Several studies have described the off-label use of roflumilast in dermatology, including a randomized controlled trial showing its usefulness in the treatment of psoriasis; case reports and small series have also reported successful outcomes in hidradenitis suppurativa, recurrent oral aphthosis, nummular eczema, lichen planus, and Behçet disease. " 01/01/2021 - "In dermatology, off-label use of tocilizumab has been reported to be efficacious in morphoea, systemic sclerosis, psoriasis, atopic dermatitis, vitiligo, graft-versus-host disease, pyoderma gangrenosum, Behcet's disease, Schnitzler's syndrome, sarcoidosis, and cutaneous adverse reactions. " 08/01/2003 - "Off-label uses for thalidomide include: aphthous stomatitis, Behçet disease, pyoderma gangrenosum, chronic discoid lupus erythematosus, systemic lupus erythematosus, lichen planus, prurigo nodularis and sarcoidosis. " 04/01/2013 - "Over the past decade, the off-label use of biologic agents such as TNF-α antagonists, including infliximab and adalimumab, has improved the treatment armamentarium for refractory immune-mediated uveitis, with particular success in Behçet disease-associated uveitis. "