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:

Networked: 148 relevant articles (13 outcomes, 12 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Erythema Nodosum
2.	Arthritis (Polyarthritis)
3.	Uveitis
4.	Retinal Vasculitis
5.	HIV Infections (HIV Infection)

Experts

1.	Hatemi, Gulen: 2 articles (10/2013 - 09/2012)
2.	Yang, Peizeng: 2 articles (06/2011 - 07/2009)
3.	Yazici, H: 2 articles (07/2007 - 12/2004)
4.	Lapadula, G: 2 articles (01/2005 - 07/2004)
5.	Şahin, Alparslan: 1 article (09/2015)
6.	Cinar, Yasin: 1 article (09/2015)
7.	Cingü, Abdullah Kürşat: 1 article (09/2015)
8.	Yüksel, Hatice: 1 article (09/2015)
9.	Kaya, Savaş: 1 article (09/2015)
10.	Türkcü, Fatih Mehmet: 1 article (09/2015)

Drugs and Biologics

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

1.	Thalidomide (Thalomid)FDA Link 06/01/1985 - "A 14-year-old girl with Behcet's syndrome showed a consistent decrease in Ia+ cells throughout her three-month course of thalidomide therapy. " 03/27/1982 - "[Treatment of Behcet's syndrome with thalidomide]." 11/01/1984 - "[Speculative ameloriation of arthritis in Behcet disease with thalidomide. " 05/01/2000 - "In controlled clinical trials, thalidomide has proven effective in the treatment of erythema nodosum leprosum, oral and oesophageal aphthous ulceration associated with advanced HIV infection and oral ulceration associated with Behcet's syndrome. " 11/01/2003 - "Thalidomide is an effective treatment for several inflammatory and autoimmune disorders including erythema nodosum leprosum, Behcet's syndrome, discoid lupus erythematosus, and Crohn's disease. "
2.	ColchicineFDA LinkGeneric 11/01/1979 - "Colchicine may be effective in controlling cutaneous necrotizing vasculitis and Behcet's syndrome through its effect on polymorphnuclear leukocyte function." 08/01/2014 - "Colchicine has been discovered to inhibit many inflammatory processes such as gout, familial Mediterranean fever, pericarditis and Behcet disease. " 11/01/1998 - "Colchicine is an alkaloid drug commonly used in familial Mediterranean fever (FMF), gout, Behcet's syndrome, psoriasis and Sweet's syndrome. " 10/01/1998 - "Colchicine is used in the treatment of gouty arthritis, familial Mediterranean fever, amyloidosis, Behcet disease and dermatoses. " 03/01/2013 - "Colchicine-induced rhabdomyolysis caused by interaction with clarithromycin in a patient with Behcet disease."
3.	SteroidsIBA 03/01/2015 - "the systemic steroids represent the first line treatment in the majority of the noninfectious uveitis, except some specific indications as the Behcet disease. " 02/01/2014 - "A 23-year-old-male patient with Behcet Disease had clinically inactive uveitis with topical steroids, oral azathiopurine and cyclosporine until he developed a retinal infiltrate in the left eye which evolved into a full-thickness macular hole during the follow-up. " 01/01/2000 - "The main results were the lack of efficacy of some of the classic treatments for Behcet's syndrome, including colchicine, cyclophosphamide and steroids for eye involvement, azapropazone and colchicine for arthritis and acyclovir, colchicine and topical interpheron for aphthas. "
4.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 07/01/2004 - "Purpose of this study was to evaluate the efficacy and safety of TNFalpha blocking agents in patients with seronegative spondylo-arthropathies (SNSA) and Behcet disease (BD) associated relapsing uveitis. " 11/01/2002 - "In addition, an increasing number of case reports indicates that anti-TNFalpha agents are of value in the treatment of other skin diseases, such as pyoderma gangrenosum and Behcet's syndrome, and in the treatment of graft-versus-host disease." 01/01/2005 - "To evaluate the efficacy of the anti-TNFα (Tumor Necrosis Factor alpha) drugs in a variety of pathologic conditions such as psoriatic arthritis, undifferentiated entheso-arthritides, chronic inflammatory bowel diseases, adult onset Still's disease, Behcet disease. " 06/01/2010 - "We report a patient with an overlap of relapsing polychondritis and Behcet disease whose cartilaginous inflammation and genital lesions were refractory to corticosteroids and immunosuppressants, but fully remitted with the tumor necrosis factor-alpha inhibitor, infliximab. "
5.	AntigensIBA 07/01/2008 - "One patient with Behcet disease and positive for human leukocyte antigen (HLA)-B51 has not improved after HSCT. " 06/01/1979 - "Lymphoproliferative responses induced by streptococcal antigens in recurrent aphthous stomatitis and Behcet's syndrome." 12/01/2006 - "These results indicate the feasibility of using peptide screening for antigen discovery in central nervous system inflammatory diseases of unknown etiology, such as multiple sclerosis, neurosarcoidosis, or Behcet's syndrome." 05/01/2005 - "(1) The hearing and vestibular disturbances in Behcet's syndrome is more prevalent than previously recognized; (2) Hearing loss in high frequencies in Behcet's patients is an indicator of cochlear involvement in this disease; (3) There is a higher prevalence of central vestibular syndrome in Behcet's patients than it was thought before; (4) HLA-B51 antigen may be able to be a prognostic factor for sensorineural hearing loss in Behcet's patients." 03/01/1993 - "22 patients suffering from Birdshot retinochoroidopathy (10), idiopathic vasculitis (6) and Behcet disease (6) and treated by cyclosporine were tested at regular intervals during 11 to 38 months by the lymphocyte stimulation test (LST) and basophil degranulation test (HBDT) with S retinal antigen. "
6.	Interleukin-6 (Interleukin 6)IBA 01/01/2014 - "Polymorphism of interleukin 6 -174 G/C in Behcet disease: case series and review of literature." 07/01/2012 - "Intravitreal MTX was associated with a significant reduction of IL-6 and IL-8 levels and was effective and well tolerated even in steroid responders with refractory retinal vasculitis due to Behcet disease." 05/01/2011 - "Patients with Behcet disease revealed significant increase in peripheral Th17 related cytokines [IL-17 (13.21 ± 4.20) ng/L, IL-6 (6.37 ± 6.51) ng/L, TGF-β1 (4477.20 ± 2146.60) ng/L, IL-23 (10.86 ± 7.86) ng/L] levels (t = -3.61, -2.33, -2.15 and -3.18; P < 0.05) and obvious decrease in Tregs related cytokines [IL-10 (6.27 ± 5.56) ng/L] (t = 5.95, P < 0.05), compared with normal controls. " 07/01/2012 - "Increased intraocular levels of IL-6, IL-8, vascular endothelial growth factor, and monocyte chemotactic protein 1 may be responsible for refractory retinal vasculitis in Behcet disease. "
7.	Prednisone (Sone)FDA LinkGeneric 09/01/1990 - "After the surgical excision of the mediastinal mass (January, 1989) until now, Behcet's syndrome is in complete remission with a low dosage of prednisone." 01/01/2011 - "On receiving Immunosuppressive treatment for Behcet disease with prednisone and azathioprine over one year the pulmonary arterial aneurysms were completely resolved and the patient was discharged from the hospital albeit with persistent hypoxic brain injury from cardiac arrest."
8.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 09/01/1996 - "This is the first report to show prothrombotic tendency in a relapse of Behcet disease and beneficial effect of cyclophosphamide therapy on haemostatic abnormalities. " 01/01/2000 - "The main results were the lack of efficacy of some of the classic treatments for Behcet's syndrome, including colchicine, cyclophosphamide and steroids for eye involvement, azapropazone and colchicine for arthritis and acyclovir, colchicine and topical interpheron for aphthas. "
9.	Interleukin-8 (Interleukin 8)IBA 07/01/2012 - "Intravitreal MTX was associated with a significant reduction of IL-6 and IL-8 levels and was effective and well tolerated even in steroid responders with refractory retinal vasculitis due to Behcet disease." 07/01/2012 - "The aqueous humor levels of interleukin (IL) -6, IL-8, vascular endothelial growth factor, and monocyte chemotactic protein 1 were significantly higher in patients with Behcet disease compared with controls. " 07/01/2012 - "Increased intraocular levels of IL-6, IL-8, vascular endothelial growth factor, and monocyte chemotactic protein 1 may be responsible for refractory retinal vasculitis in Behcet disease. "
10.	Platelet Aggregation Inhibitors (Antiplatelet Drugs)IBA 07/01/1992 - "Of the 8 patients showing Raynaud's syndrome, 4 patients of scleroderma and 1 patient of multiple myeloma showed no improvement of finger blood flow in the cold exposed hand after 2 weeks of pharmacological therapy, but 1 patient of mixed connective tissue disease, 1 patient of Behcet's syndrome and 1 patient of SLE showed much improved finger blood flow after combined administration of vasodilator, calcium channel blockers and antiplatelet drugs. "

Therapies and Procedures

1.	Transplants (Transplant) 07/01/2008 - "Three patients (one with Behcet disease, one with Sjögren syndrome, and one with Wegener granulomatosis) entered a sustained remission at 6, 6 and 24 months, respectively, after transplant. " 06/02/2009 - "IMAGE CARDIO MED: Oppression of left main trunk due to pseudoaneurysm with graft detachment in patients with Behcet disease previously treated by the Bentall procedure."
2.	Autologous Transplantation 01/01/2007 - "Prolonged remission of neuro-Behcet disease following autologous transplantation."
3.	Plasma Exchange 09/01/1983 - "Seven patients with Behcet's syndrome were given plasma exchanges (PE). " 05/15/1982 - "[Value of plasma exchange in the treatment of Behcet's syndrome and its eye manifestations]."
4.	Drug Therapy (Chemotherapy) 01/01/2000 - "Pharmacotherapy for Behcet's syndrome." 07/01/2008 - "Four patients with refractory SV (two with neurovascular Behcet disease, one with neurovascular Sjögren syndrome, and one with Wegener granulomatosis) were involved in an Institutional Review Board (IRB) and US Food and Drug Administration (FDA) approved phase I clinical trial of high dose chemotherapy and autologous HSCT. "
5.	Immunomodulation 07/01/2007 - "Adamantiades-Behcet disease with retinal involvement is now considered an absolute indication for systemic immunomodulatory therapy. "