MCID: BHC003
MIFTS: 68

Behcet Syndrome

Categories: Rare diseases, Neuronal diseases, Eye diseases, Cardiovascular diseases, Nephrological diseases, Bone diseases, Skin diseases

Aliases & Classifications for Behcet Syndrome

MalaCards integrated aliases for Behcet Syndrome:

Name: Behcet Syndrome 53 12 72 49 24 41 37 69 49
Behcet's Disease 37 12 49 50 14
Behcet's Syndrome 12 49 24 40
Behcet Disease 53 24 36 51
Behçet Disease 49 24 55
Adamantiades-Behcet Disease 12 24
Triple Symptom Complex 12 24
Bd 53 49
Behcet Triple Symptom Complex 24
Old Silk Route Disease 24
Malignant Aphthosis 24
Behcet Disease; Bd 53
Behçet's Syndrome 49
Behet's Syndrome 12
Behçet's Disease 49
Behçet’s Disease 49

Characteristics:

Orphanet epidemiological data:

55
behçet disease
Inheritance: Multigenic/multifactorial; Prevalence: 1-5/10000 (Japan),>1/1000 (Turkey); Age of onset: Adolescent,Adult,Childhood; Age of death: adult;

OMIM:

53
Inheritance:
familial cases reported, but probably not mendelian


Classifications:



Summaries for Behcet Syndrome

NINDS : 50 Behcet's disease is a rare, chronic inflammatory disorder. The cause of Behcet's disease is unknown, but current research suggests that both genetic and  environmental factors play a role.  Behcet's disease generally begins when individuals are in their 20s or 30s, although it can happen at any age. It tends to occur more often in men than in women. Symptoms of Behcet's disease include recurrent ulcers in the mouth (resembling canker sores) and on the genitals, and eye inflammation. The disorder may also cause various types of skin lesions, arthritis, bowel inflammation, meningitis (inflammation of the membranes of the brain and spinal cord), and cranial nerve palsies. Behcet's is a multi-system disease; it may involve all organs and affect the central nervous system, causing memory loss and impaired speech, balance, and movement. The effects of the disease may include blindness, stroke, swelling of the spinal cord, and intestinal complications. The disease is common in the Middle East, particularly in Turkey, and in Far Eastern nations such as Japan and Korean, but is less common in the United States.

MalaCards based summary : Behcet Syndrome, also known as behcet's disease, is related to aphthous stomatitis and binswanger's disease, and has symptoms including ataxia, arthralgia and fatigue. An important gene associated with Behcet Syndrome is MEFV (MEFV, Pyrin Innate Immunity Regulator), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Methylprednisolone and Prednisolone have been mentioned in the context of this disorder. Affiliated tissues include eye, skin and brain, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

NIH Rare Diseases : 49 Behçet disease is a chronic multisystem inflammatory disorder characterized by ulcers affecting the mouth and genitals, various skin lesions, and abnormalities affecting the eyes. In some people, the disease also results in arthritis (swollen, painful, stiff joints), skin problems, and inflammation of the digestive tract, brain, and spinal cord. Although it can happen at any age, symptoms generally begin when individuals are in their 20s or 30s. The disease is common in Japan, Turkey and Israel, and less common in the United States. The exact cause of Behçet disease is still unknown.  Treatment is symptomatic and supportive. Research is being conducted on the use of interferon-alpha and with agents which inhibit tumor necrosis factor (TNF) for the treatment of Behçet disease. Behçet disease is a lifelong disorder that comes and goes. Spontaneous remission over time is common for individuals with Behçet disease but permanent remission of symptoms has not been reported. Last updated: 11/2/2016

MedlinePlus : 40 Behcet's syndrome is a disease that involves vasculitis, which is inflammation of the blood vessels. It causes problems in many parts of the body. The most common symptoms are Sores in the mouth Sores on the sex organs Other skin sores Swelling of parts of the eye Pain, swelling and stiffness of the joints More serious problems can include meningitis, blood clots, inflammation of the digestive system and blindness. Doctors aren't sure what causes Behcet's. It is rare in the United States, but is common in the Middle East and Asia. It mainly affects people in their 20s and 30s. Diagnosing Behcet's can take a long time, because symptoms may come and go, and it may take months or even years to have all of the symptoms. There is no cure. Treatment focuses on reducing pain and preventing serious problems. Most people can control symptoms with treatment. NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Disease Ontology : 12 A vasculitis that is characterized by an autoimmune mediated inflammation of the blood vessels throughout the body leading to ulcerations on the mouth and sometimes the genitals, notorious for causing hypopyon uveitis.

Wikipedia : 72 Behçet\'s disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The... more...

Description from OMIM: 109650

Related Diseases for Behcet Syndrome

Diseases related to Behcet Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 129)
# Related Disease Score Top Affiliating Genes
1 aphthous stomatitis 30.7 HLA-B IL10 MEFV TNF
2 binswanger's disease 11.2
3 dementia - subcortical 11.2
4 subcortical arteriosclerotic encephalopathy 11.2
5 intellectual disability - athetosis - microphthalmia 11.2
6 boomerang dysplasia 11.2
7 diamond-blackfan anemia 1 11.1
8 spondyloepiphyseal dysplasia-brachydactyly and distinctive speech 11.1
9 retinal vasculitis 11.1
10 magic syndrome 11.1
11 diamond-blackfan anemia 11.0
12 behcet's syndrome arthropathy 10.9
13 psoriatic juvenile idiopathic arthritis 10.9 MEFV NOD2 PSTPIP1
14 vasculitis 10.9
15 inflammatory myopathy with abundant macrophages 10.9 MEFV TNFRSF1A
16 intermittent hydrarthrosis 10.9 MEFV TNFRSF1A
17 pouchitis 10.9 IL10 NOD2 TLR4
18 microscopic polyangiitis 10.9 HLA-B IL10 TNF
19 brachydactyly, type e1 10.9
20 bipolar i disorder 10.9
21 punctate inner choroidopathy 10.9 IL10 TNF
22 relapsing fever 10.9 MEFV TNF TNFRSF1A
23 periodic fever, familial, autosomal dominant 10.9 MEFV TNF TNFRSF1A
24 blau syndrome 10.9 MEFV NOD2 TNF
25 intermediate uveitis 10.9 HLA-B IL10 TNF
26 pyoderma gangrenosum 10.9 MEFV NOD2 PSTPIP1 TNF
27 pyoderma 10.9 MEFV NOD2 PSTPIP1 TNF
28 hematopoietic stem cell transplantation 10.9 HLA-B IL10 TNF
29 panuveitis 10.9 HLA-B IL10 TNF
30 transient hypogammaglobulinemia 10.9 IL10 LTA TNF
31 mucocutaneous leishmaniasis 10.9 IL10 LTA TNF
32 palindromic rheumatism 10.9 MEFV TNF TNFRSF1A
33 acute graft versus host disease 10.9 IL10 NOD2 TNF
34 anterior uveitis 10.9 HLA-B TLR4 TNF
35 spondyloarthropathy 1 10.9 HLA-B NOD2 TNF TNFRSF1A
36 brucellosis 10.9 IL10 MEFV TLR4 TNF
37 scrub typhus 10.9 IL10 TLR4 TNF
38 alveolar echinococcosis 10.9 HLA-B TLR4 TNF
39 uveitis 10.9 HLA-B IL10 NOD2 TNF
40 q fever 10.9 IL10 TLR4 TNF
41 complex regional pain syndrome 10.9 HLA-B IL10 TNF
42 cytomegalovirus infection 10.9 FAS IL10 TNF
43 post-transplant lymphoproliferative disease 10.8 IL10 LTA TNF TNFRSF1A
44 primary bacterial infectious disease 10.8 IL10 MEFV TLR4 TNF
45 lymphadenitis 10.8 IL10 NOD2 TLR4 TNF
46 epstein-barr virus-associated gastric carcinoma 10.8 FAS IL10 TNF
47 tropical endomyocardial fibrosis 10.8 IL10 TNF
48 spondylarthropathy 10.8 LTA TLR4 TNF
49 familial mediterranean fever 10.8 MEFV NOD2 PSTPIP1 TNF TNFRSF1A
50 idiopathic achalasia 10.8 IL10 IL23R LTA TNF

Graphical network of the top 20 diseases related to Behcet Syndrome:



Diseases related to Behcet Syndrome

Symptoms & Phenotypes for Behcet Syndrome

Symptoms via clinical synopsis from OMIM:

53
Skin:
hyperirritability
raynaud phenomenon
erythema nodosum-like eruptions
superficial thrombophlebitis
pustular skin lesions

Eyes:
uveitis
iritis
iridocyclitis
hypopyon
choreoretinitis

Mouth:
mouth ulcerations

Neuro:
brainstem syndrome
meningoencephalomyelitic syndrome
organic confusional state
schizoaffective disorder

Joints:
arthritis

Hair:
alopecia areata

GU:
genital ulcerations
epididymitis


Clinical features from OMIM:

109650

Human phenotypes related to Behcet Syndrome:

55 31 (show top 50) (show all 78)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 ataxia 55 31 occasional (7.5%) Occasional (29-5%) HP:0001251
2 arthralgia 55 31 frequent (33%) Frequent (79-30%) HP:0002829
3 fatigue 55 31 hallmark (90%) Very frequent (99-80%) HP:0012378
4 myalgia 55 31 hallmark (90%) Very frequent (99-80%) HP:0003326
5 fever 55 31 hallmark (90%) Very frequent (99-80%) HP:0001945
6 seizures 55 31 occasional (7.5%) Occasional (29-5%) HP:0001250
7 vertigo 55 31 occasional (7.5%) Occasional (29-5%) HP:0002321
8 hemoptysis 55 31 occasional (7.5%) Occasional (29-5%) HP:0002105
9 photophobia 55 31 hallmark (90%) Very frequent (99-80%) HP:0000613
10 abdominal pain 55 31 frequent (33%) Frequent (79-30%) HP:0002027
11 nausea and vomiting 55 31 hallmark (90%) Very frequent (99-80%) HP:0002017
12 hemiparesis 55 31 frequent (33%) Frequent (79-30%) HP:0001269
13 abnormal pyramidal signs 55 31 occasional (7.5%) Occasional (29-5%) HP:0007256
14 gait disturbance 55 31 frequent (33%) Frequent (79-30%) HP:0001288
15 hyperreflexia 55 31 occasional (7.5%) Occasional (29-5%) HP:0001347
16 developmental regression 55 31 occasional (7.5%) Occasional (29-5%) HP:0002376
17 encephalitis 55 31 occasional (7.5%) Occasional (29-5%) HP:0002383
18 cataract 55 31 occasional (7.5%) Occasional (29-5%) HP:0000518
19 arthritis 55 31 hallmark (90%) Very frequent (99-80%) HP:0001369
20 splenomegaly 55 31 occasional (7.5%) Occasional (29-5%) HP:0001744
21 increased intracranial pressure 55 31 occasional (7.5%) Occasional (29-5%) HP:0002516
22 aseptic necrosis 55 31 occasional (7.5%) Occasional (29-5%) HP:0010885
23 malabsorption 55 31 occasional (7.5%) Occasional (29-5%) HP:0002024
24 blindness 55 31 occasional (7.5%) Occasional (29-5%) HP:0000618
25 renal insufficiency 55 31 occasional (7.5%) Occasional (29-5%) HP:0000083
26 retinopathy 55 31 occasional (7.5%) Occasional (29-5%) HP:0000488
27 cranial nerve paralysis 55 31 occasional (7.5%) Occasional (29-5%) HP:0006824
28 subcutaneous nodule 55 31 hallmark (90%) Very frequent (99-80%) HP:0001482
29 myocardial infarction 55 31 occasional (7.5%) Occasional (29-5%) HP:0001658
30 pulmonary embolism 55 31 occasional (7.5%) Occasional (29-5%) HP:0002204
31 abnormal blistering of the skin 55 31 frequent (33%) Frequent (79-30%) HP:0008066
32 irritability 55 31 occasional (7.5%) Occasional (29-5%) HP:0000737
33 weight loss 55 31 occasional (7.5%) Occasional (29-5%) HP:0001824
34 acne 55 31 frequent (33%) Frequent (79-30%) HP:0001061
35 venous thrombosis 55 31 frequent (33%) Frequent (79-30%) HP:0004936
36 pancreatitis 55 31 occasional (7.5%) Occasional (29-5%) HP:0001733
37 anorexia 55 31 occasional (7.5%) Occasional (29-5%) HP:0002039
38 migraine 55 31 hallmark (90%) Very frequent (99-80%) HP:0002076
39 memory impairment 55 31 occasional (7.5%) Occasional (29-5%) HP:0002354
40 cerebral ischemia 55 31 occasional (7.5%) Occasional (29-5%) HP:0002637
41 paresthesia 55 31 occasional (7.5%) Occasional (29-5%) HP:0003401
42 glomerulopathy 55 31 occasional (7.5%) Occasional (29-5%) HP:0100820
43 keratoconjunctivitis sicca 55 31 occasional (7.5%) Occasional (29-5%) HP:0001097
44 mitral regurgitation 55 31 occasional (7.5%) Occasional (29-5%) HP:0001653
45 gastrointestinal hemorrhage 55 31 frequent (33%) Frequent (79-30%) HP:0002239
46 pericarditis 55 31 occasional (7.5%) Occasional (29-5%) HP:0001701
47 endocarditis 55 31 occasional (7.5%) Occasional (29-5%) HP:0100584
48 meningitis 55 31 hallmark (90%) Very frequent (99-80%) HP:0001287
49 vasculitis 55 31 hallmark (90%) Very frequent (99-80%) HP:0002633
50 arterial thrombosis 55 31 occasional (7.5%) Occasional (29-5%) HP:0004420

UMLS symptoms related to Behcet Syndrome:


snoring, pruritus, oral manifestations, halitosis, exanthema, edema, chest pain, angina pectoris

GenomeRNAi Phenotypes related to Behcet Syndrome according to GeneCards Suite gene sharing:

25 (show top 50) (show all 52)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 10.21 IL10
2 Increased shRNA abundance (Z-score > 2) GR00366-A-101 10.21 CCR1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-103 10.21 HLA-B
4 Increased shRNA abundance (Z-score > 2) GR00366-A-105 10.21 ADA2 CCR1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-106 10.21 ADA2
6 Increased shRNA abundance (Z-score > 2) GR00366-A-107 10.21 C4A ADA2
7 Increased shRNA abundance (Z-score > 2) GR00366-A-110 10.21 HLA-B
8 Increased shRNA abundance (Z-score > 2) GR00366-A-113 10.21 C4A CCR1 TLR4 IL10
9 Increased shRNA abundance (Z-score > 2) GR00366-A-114 10.21 TLR4
10 Increased shRNA abundance (Z-score > 2) GR00366-A-118 10.21 HLA-B
11 Increased shRNA abundance (Z-score > 2) GR00366-A-120 10.21 CCR1
12 Increased shRNA abundance (Z-score > 2) GR00366-A-125 10.21 IL10
13 Increased shRNA abundance (Z-score > 2) GR00366-A-128 10.21 IL10 TLR4
14 Increased shRNA abundance (Z-score > 2) GR00366-A-130 10.21 ADA2
15 Increased shRNA abundance (Z-score > 2) GR00366-A-132 10.21 IL10
16 Increased shRNA abundance (Z-score > 2) GR00366-A-137 10.21 C4A
17 Increased shRNA abundance (Z-score > 2) GR00366-A-147 10.21 CCR1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-152 10.21 IL10
19 Increased shRNA abundance (Z-score > 2) GR00366-A-156 10.21 HLA-B
20 Increased shRNA abundance (Z-score > 2) GR00366-A-16 10.21 ADA2
21 Increased shRNA abundance (Z-score > 2) GR00366-A-161 10.21 C4A IL10
22 Increased shRNA abundance (Z-score > 2) GR00366-A-163 10.21 ADA2
23 Increased shRNA abundance (Z-score > 2) GR00366-A-169 10.21 TLR4
24 Increased shRNA abundance (Z-score > 2) GR00366-A-174 10.21 NOD2
25 Increased shRNA abundance (Z-score > 2) GR00366-A-177 10.21 NOD2
26 Increased shRNA abundance (Z-score > 2) GR00366-A-178 10.21 HLA-B
27 Increased shRNA abundance (Z-score > 2) GR00366-A-19 10.21 HLA-B C4A ADA2 NOD2
28 Increased shRNA abundance (Z-score > 2) GR00366-A-192 10.21 NOD2
29 Increased shRNA abundance (Z-score > 2) GR00366-A-196 10.21 CCR1
30 Increased shRNA abundance (Z-score > 2) GR00366-A-198 10.21 IL10
31 Increased shRNA abundance (Z-score > 2) GR00366-A-199 10.21 ADA2
32 Increased shRNA abundance (Z-score > 2) GR00366-A-210 10.21 IL10 NOD2
33 Increased shRNA abundance (Z-score > 2) GR00366-A-214 10.21 IL10
34 Increased shRNA abundance (Z-score > 2) GR00366-A-22 10.21 TLR4
35 Increased shRNA abundance (Z-score > 2) GR00366-A-25 10.21 IL10
36 Increased shRNA abundance (Z-score > 2) GR00366-A-31 10.21 HLA-B
37 Increased shRNA abundance (Z-score > 2) GR00366-A-36 10.21 CCR1 NOD2
38 Increased shRNA abundance (Z-score > 2) GR00366-A-43 10.21 C4A NOD2
39 Increased shRNA abundance (Z-score > 2) GR00366-A-47 10.21 ADA2
40 Increased shRNA abundance (Z-score > 2) GR00366-A-49 10.21 ADA2
41 Increased shRNA abundance (Z-score > 2) GR00366-A-52 10.21 ADA2 IL10
42 Increased shRNA abundance (Z-score > 2) GR00366-A-63 10.21 C4A CCR1
43 Increased shRNA abundance (Z-score > 2) GR00366-A-67 10.21 IL10
44 Increased shRNA abundance (Z-score > 2) GR00366-A-7 10.21 HLA-B
45 Increased shRNA abundance (Z-score > 2) GR00366-A-74 10.21 HLA-B
46 Increased shRNA abundance (Z-score > 2) GR00366-A-8 10.21 C4A
47 Increased shRNA abundance (Z-score > 2) GR00366-A-80 10.21 IL10 ADA2
48 Increased shRNA abundance (Z-score > 2) GR00366-A-82 10.21 CCR1 IL10
49 Increased shRNA abundance (Z-score > 2) GR00366-A-85 10.21 IL10
50 Increased shRNA abundance (Z-score > 2) GR00366-A-9 10.21 ADA2

MGI Mouse Phenotypes related to Behcet Syndrome:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.25 MEFV IL10 NOD2 TLR4 PSTPIP1 IL12A
2 immune system MP:0005387 10.17 MEFV IL10 NOD2 TLR4 PSTPIP1 IL12A
3 homeostasis/metabolism MP:0005376 10.15 FAS TNFRSF1A MEFV NOD2 IL10 STAT4
4 digestive/alimentary MP:0005381 10.03 FAS TNFRSF1A NOD2 IL10 TLR4 IL23R
5 liver/biliary system MP:0005370 9.86 MEFV IL10 TLR4 CCR1 TNF LTA
6 neoplasm MP:0002006 9.7 FAS TNFRSF1A IL10 TLR4 IL12A IL23R
7 respiratory system MP:0005388 9.56 IL10 TLR4 IL12A CCR1 TNF LTA
8 skeleton MP:0005390 9.28 FAS TNFRSF1A MEFV NOD2 IL10 TLR4

Drugs & Therapeutics for Behcet Syndrome

Drugs for Behcet Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 83)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methylprednisolone Approved, Vet_approved Phase 4,Phase 3,Phase 2 83-43-2 6741
2
Prednisolone Approved, Vet_approved Phase 4,Phase 3,Phase 2 50-24-8 5755
3
Fluocinolone Acetonide Approved, Investigational, Vet_approved Phase 4 67-73-2 6215
4 Antiemetics Phase 4
5 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2
6 Antineoplastic Agents, Hormonal Phase 4
7 Autonomic Agents Phase 4
8 Gastrointestinal Agents Phase 4,Phase 3,Phase 1,Phase 2
9 glucocorticoids Phase 4
10 Hormone Antagonists Phase 4
11 Hormones Phase 4
12 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
13 Methylprednisolone acetate Phase 4,Phase 3,Phase 2
14 Methylprednisolone Hemisuccinate Phase 4,Phase 3,Phase 2
15 Neuroprotective Agents Phase 4
16 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 2
17 Prednisolone acetate Phase 4,Phase 3,Phase 2
18 Prednisolone hemisuccinate Phase 4,Phase 3,Phase 2
19 Prednisolone phosphate Phase 4,Phase 3,Phase 2
20 Protective Agents Phase 4
21 Pharmaceutical Solutions Phase 4,Phase 3,Phase 1
22 Omega 3 Fatty Acid Nutraceutical Phase 4
23
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
24
Adalimumab Approved Phase 3 331731-18-1 16219006
25
Infliximab Approved Phase 3,Phase 1,Phase 2 170277-31-3
26
Apremilast Approved, Investigational Phase 3,Phase 2 608141-41-9 11561674
27
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 50-18-0, 6055-19-2 2907
28 Antifungal Agents Phase 3
29 Anti-Infective Agents Phase 3,Phase 2
30 Antirheumatic Agents Phase 3,Phase 2,Phase 1,Early Phase 1
31 Antiviral Agents Phase 3,Phase 2
32 Calcineurin Inhibitors Phase 3
33 Cyclosporins Phase 3
34 Dermatologic Agents Phase 3,Phase 1,Phase 2
35 Immunosuppressive Agents Phase 3,Phase 2,Early Phase 1
36 Interferon-alpha Phase 3,Phase 2
37 interferons Phase 3,Phase 2
38 Analgesics Phase 3,Phase 2
39 Analgesics, Non-Narcotic Phase 3,Phase 2
40 Anti-Inflammatory Agents, Non-Steroidal Phase 3,Phase 2
41 Antibodies Phase 3,Phase 2
42 Antibodies, Monoclonal Phase 3,Phase 2
43 Immunoglobulins Phase 3,Phase 2
44 Angiogenesis Inhibitors Phase 3,Phase 2
45 Angiogenesis Modulating Agents Phase 3,Phase 2
46 Anti-Bacterial Agents Phase 3,Phase 2
47 Alkylating Agents Phase 3
48
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
49
Colchicine Approved Phase 2 64-86-8 2833 6167
50
Azathioprine Approved Phase 2 446-86-6 2265

Interventional clinical trials:

(show top 50) (show all 63)

# Name Status NCT ID Phase Drugs
1 The Efficacy of Methylprednisolone in the Treatment of Patients With Ocular Involvement in Behcet's Disease Unknown status NCT01306955 Phase 4 methylorednisolone
2 Flucinolone Acetonide Implant for Treating Refractory Ocular Behcet's Disease Unknown status NCT00720928 Phase 4 flucinolone acetonide
3 Saline vs. Lactated Ringers for Emergency Department IV Fluid Resuscitation Completed NCT03133767 Phase 4 Lactated Ringer Solution;Normal Saline 0.9% Infusion Solution Bag
4 Trial to Study the Effects of Supplementary Omega-3 on Serum C-Reactive Protein Levels Terminated NCT00578578 Phase 4
5 Interferon-alpha2a Versus Cyclosporin A for Severe Ocular Behcet`s Disease (INCYTOB) Unknown status NCT00167583 Phase 3 Cyclosporin A;Interferon-alpha2a
6 Clinical Study of TA-650 in Patients With Behcet's Disease (BD) With Special Lesions Completed NCT01532570 Phase 3 TA-650
7 A Study of Adalimumab in Japanese Subjects With Intestinal Behçet's Disease Completed NCT01243671 Phase 3
8 Phase III Study in Refractory Behcet's Disease Completed NCT00995709 Phase 3 AIN457;AIN457;Placebo
9 Efficacy of Humira in Behcet Patients With Arthritis Completed NCT01497717 Phase 3 Adalimumab (Humira)
10 Interferon α2a Versus Cyclosporine for Refractory Behçet`s Disease Uveitis Recruiting NCT03209219 Phase 3 Interferon Alfa-2A;Cyclosporine Pill
11 A Study to Evaluate Efficacy and Safety of Infliximab in Participant With Moderate-to-Severe Refractory Intestinal Behcet's Disease Active, not recruiting NCT02505568 Phase 3 Infliximab
12 A Phase 3 Randomized, Double-blind Study to Evaluate the Efficacy and Safety of Apremilast (CC-10004) in Subjects With Active Behcet's Disease Active, not recruiting NCT02307513 Phase 3 Apremilast (CC-10004);Placebo
13 Induction Therapy With Anti-TNFα vs Cyclophosphamide in Severe Behçet Disease Not yet recruiting NCT03371095 Phase 3 Infliximab;Cyclophosphamide
14 Thalidomide Versus Infliximab in New Onset Crohn's Disease With Poor Prognostic Factors Not yet recruiting NCT03221166 Phase 3 Thalidomide;Infliximab
15 Efficacy and Safety Study of Gevokizumab to Treat Behcet's Disease Uveitis Terminated NCT02258867 Phase 3 Placebo;Gevokizumab
16 Efficacy of Gevokizumab in the Treatment of Patients With Behçet's Disease Uveitis (EYEGUARD™-B) Terminated NCT01965145 Phase 3 Gevokizumab;Placebo
17 Long-term Safety of Gevokizumab in the Treatment of Patients With Chronic Non-infectious Uveitis (EYEGUARD-X) Terminated NCT02375685 Phase 3
18 An Open-label, Rollover Study Providing Continued Dosing of Gevokizumab in Order to Assess Long-term Gevokizumab Safety Data Terminated NCT02258854 Phase 3 Dose 2 gevokizumab
19 Colchicine Randomized Double-Blind Controlled Crossover Study in Behcet's Disease Completed NCT00700297 Phase 2 Colchicine;Placebo
20 A Study to Evaluate the Efficacy and Safety of Apremilast (CC-10004) in the Treatment of Behçet Disease Completed NCT00866359 Phase 2 Apremilast (CC-10004);Placebo
21 Rituximab for the Treatment of Severe Ocular Manifestations of Behcet's Disease Completed NCT00664599 Phase 2 Rituximab;Cytotoxic Combination
22 Intravitreal Infliximab in Refractory Uveitis in Behcet's Disease: A Safety and Efficacy Clinical Study Completed NCT02620618 Phase 1, Phase 2 Intravitreal Infliximab
23 Low Dose Interferon Alpha Treatment for Oral Ulcers of Behcet's Disease Completed NCT00483184 Phase 2
24 HAT in Eye Complications of Behcet's Disease Completed NCT00001865 Phase 2 Daclizumab
25 Anakinra for Behcet s Disease Completed NCT01441076 Phase 1, Phase 2 Anakinra
26 Ilaris (Canakinumab) in the Schnitzler Syndrome Completed NCT01245127 Phase 2 Ilaris
27 Efficacy and Safety Study of Two Doses of Apremilast (CC-10004) In Japanese Subjects With Moderate-To-Severe Plaque-Type Psoriasis Completed NCT01988103 Phase 2 Apremilast;Apremilast;Placebo
28 Efficacy and Safety of Ustekinumab, a Human Monoclonal Anti-IL-12/IL-23 Antibody, in Patients With Behçet Disease Recruiting NCT02648581 Phase 2 Subcutaneous Ustekinumab
29 1 Year Canakinumab Treatment in Behcet's Disease Patients With Neurologic or Vascular Involvement Recruiting NCT02756650 Phase 2 drug administration
30 Induction of Regulatory t Cells by Low Dose il2 in Autoimmune and Inflammatory Diseases Recruiting NCT01988506 Phase 2 Interleukin 2
31 Tocilizumab for the Treatment of Behcet's Syndrome Terminated NCT01693653 Phase 2 Tocilizumab
32 A Pilot Study of XOMA 052 in Familial Cold Autoinflammatory Syndrome / Muckle-Wells Syndrome and Behcet's Disease Withdrawn NCT01211977 Phase 1, Phase 2 XOMA 052
33 Stem Cell Transplantation for the Treatment of Knee Osteoarthritis Unknown status NCT00550524 Phase 1
34 Stem Cell Transplantation in Ocular Lesions of Behcet's Disease Terminated NCT00550498 Phase 1
35 Hematopoietic Stem Cell Support in Vasculitis Terminated NCT00278512 Phase 1
36 Etanercept: Single Blind Control Study in Ocular Manifestations of Behcet's Disease Unknown status NCT00931957 Etanercept, Methotrexate, Prednisolone
37 The Serum Angiotensin Converting Enzyme and Lysozyme Levels in Patients Non-infectious and Infectious Uveitis Unknown status NCT02627209
38 Psychological Symptoms in Patients With Behcet's Disease by SCL90-R Completed NCT00699985
39 Special Investigation in Patients With Intestinal Behcet's Disease (All Case Investigation) Completed NCT01960790
40 Behçet's Disease and Eosinophil Cationic Protein Completed NCT01584778
41 MEVALONATE KINASE GENE MUTATIONS AND THEIR CLINICAL CORRELATIONS IN BEHÇET'S DISEASE Completed NCT01780363
42 The Relationship Between Serum Levels of Angiogenin, bFGF, VEGF and Ocular Involvement in Patients With Behçet's Disease Completed NCT01720628
43 Educational Needs of Patients With Systemic Vasculitis Completed NCT02190929
44 Biological Markers in Retinal Vasculitis Completed NCT00050492
45 Uveitis Gene-Expression Profiling Completed NCT00874471
46 Impact of Vasculitis on Employment and Income Completed NCT02476292
47 Vasculitis Illness Perception (VIP) Study Completed NCT02190916
48 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
49 Steroid-Induced Osteoporosis in the Pediatric Population - Canadian Incidence Study Completed NCT01663129
50 Evaluation and Treatment of Patients With Inflammatory Eye Diseases Completed NCT00006188

Search NIH Clinical Center for Behcet Syndrome

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Behcet Syndrome cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Behcet Syndrome:
Bone marrow-derived stem cells for treatment of ocular lesions Behcet's disease
Embryonic/Adult Cultured Cells Related to Behcet Syndrome:
Bone marrow-derived stem cells PMIDs: 21199472 23773637

Cochrane evidence based reviews: behcet syndrome

Genetic Tests for Behcet Syndrome

Anatomical Context for Behcet Syndrome

MalaCards organs/tissues related to Behcet Syndrome:

38
Eye, Skin, Brain, Spinal Cord, Bone, Neutrophil, T Cells

Publications for Behcet Syndrome

Articles related to Behcet Syndrome:

# Title Authors Year
1
Delayed Vasospasm after Aneurysmal Subarachnoid Hemorrhage in Behcet Syndrome. ( 27114963 )
2016
2
Clinical and Ultrasonographic Evaluation of Lower-extremity Vein Thrombosis in Behcet Syndrome: An Observational Study. ( 26554787 )
2015
3
JAK2 (V617F) mutation is not associated with thrombosis in Behcet syndrome. ( 22203033 )
2012
4
Summary of workshop on recurrent aphthous stomatitis and Behcet syndrome. ( 281401 )
1978

Variations for Behcet Syndrome

ClinVar genetic disease variations for Behcet Syndrome:

6 (show all 11)
# Gene Variation Type Significance SNP ID Assembly Location
1 NOD2 NM_022162.2(NOD2): c.2446G> A single nucleotide variant Pathogenic rs886040969 GRCh37 Chromosome 16, 50746268: 50746268
2 TNFRSF1A NM_001065.3(TNFRSF1A): c.463C> T single nucleotide variant Pathogenic rs886039866 GRCh37 Chromosome 12, 6442542: 6442542
3 NOD2 NM_022162.2(NOD2): c.2197G> T single nucleotide variant Pathogenic rs746055479 GRCh38 Chromosome 16, 50712108: 50712108
4 NOD2 NM_022162.2(NOD2): c.241C> G single nucleotide variant Pathogenic rs34936594 GRCh37 Chromosome 16, 50733566: 50733566
5 NOD2 NM_022162.2(NOD2): c.1045C> T (p.Leu349Phe) single nucleotide variant Pathogenic rs752615209 GRCh38 Chromosome 16, 50710956: 50710956
6 PSTPIP1 NM_003978.4(PSTPIP1): c.865G> C (p.Asp289His) single nucleotide variant Pathogenic rs774164456 GRCh38 Chromosome 15, 77032888: 77032888
7 ADA2 NM_001282225.1(ADA2): c.145C> T (p.Arg49Trp) single nucleotide variant Pathogenic rs199614299 GRCh38 Chromosome 22, 17209533: 17209533
8 ADA2 NM_001282225.1(ADA2): c.740C> T (p.Ala247Val) single nucleotide variant Pathogenic rs750868279 GRCh38 Chromosome 22, 17203576: 17203576
9 MEFV NM_000243.2(MEFV): c.1211A> G (p.His404Arg) single nucleotide variant Pathogenic rs755659290 GRCh38 Chromosome 16, 3249480: 3249480
10 MEFV NM_000243.2(MEFV): c.1099C> G (p.Leu367Val) single nucleotide variant Pathogenic rs1057519328 GRCh38 Chromosome 16, 3249592: 3249592
11 MEFV NM_000243.2(MEFV): c.332G> A (p.Gly111Glu) single nucleotide variant Pathogenic rs751454741 GRCh37 Chromosome 16, 3304736: 3304736

Copy number variations for Behcet Syndrome from CNVD:

7
# CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 241985 8 6835170 6856724 Copy number DEFA1 Behcet''s disease

Expression for Behcet Syndrome

Search GEO for disease gene expression data for Behcet Syndrome.

Pathways for Behcet Syndrome

Pathways related to Behcet Syndrome according to GeneCards Suite gene sharing:

(show all 46)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.95 ADA2 C4A CCR1 ERAP1 HLA-B IL10
2
Show member pathways
13.82 CCR1 FAS IL10 IL12A IL23R LTA
3
Show member pathways
13.67 CCR1 FAS IL10 IL12A IL23R LTA
4
Show member pathways
13.37 CCR1 HLA-B IL10 IL12A IL23R LTA
5
Show member pathways
13.37 CCR1 FAS IL10 IL12A IL23R LTA
6
Show member pathways
13.31 CCR1 FAS IL10 IL12A IL23R LTA
7
Show member pathways
12.79 FAS HLA-B IL12A LTA TLR4 TNF
8
Show member pathways
12.64 FAS IL12A LTA TLR4 TNF TNFRSF1A
9
Show member pathways
12.6 C4A FAS HLA-B IL10 IL12A KLRC4
10 12.57 FAS IL10 STAT4 TNF TNFRSF1A
11
Show member pathways
12.55 FAS IL10 IL12A TLR4 TNF TNFRSF1A
12
Show member pathways
12.54 IL10 IL12A IL23R NOD2 STAT4 TLR4
13
Show member pathways
12.5 CCR1 FAS HLA-B STAT4 TLR4 TNF
14
Show member pathways
12.41 FAS IL10 PSTPIP1 TNF
15 12.4 HLA-B LTA TNF TNFRSF1A
16
Show member pathways
12.36 IL10 IL12A IL23R STAT4
17
Show member pathways
12.28 IL10 IL12A TLR4 TNF TNFRSF1A
18
Show member pathways
12.24 FAS STAT4 TLR4 TNF TNFRSF1A
19
Show member pathways
12.14 HLA-B IL10 IL12A LTA TNF
20 12.13 IL10 IL12A NOD2 TLR4 TNF TNFRSF1A
21 12 IL10 IL12A IL23R TNF
22 11.94 FAS LTA NOD2 TNF TNFRSF1A
23 11.92 IL10 IL12A TLR4 TNF
24 11.91 LTA TLR4 TNF TNFRSF1A
25 11.87 CCR1 IL10 IL12A IL23R STAT4
26
Show member pathways
11.83 IL23R STAT4 TLR4
27
Show member pathways
11.83 FAS LTA TNF TNFRSF1A
28 11.73 IL10 IL12A TNF
29 11.71 TLR4 TNF TNFRSF1A
30 11.71 IL10 LTA TLR4 TNF
31 11.67 IL10 IL12A IL23R LTA TNF
32 11.61 LTA TLR4 TNF TNFRSF1A
33 11.6 C4A IL10 IL12A TLR4 TNF
34 11.59 IL12A TLR4 TNF
35 11.55 FAS TNF TNFRSF1A
36
Show member pathways
11.54 LTA TNF TNFRSF1A
37 11.54 IL10 IL12A TLR4 TNF
38 11.52 IL12A TLR4 TNF
39 11.48 FAS TNF TNFRSF1A
40 11.37 IL10 TLR4 TNF
41 11.34 FAS IL10 IL12A TNF
42 11.24 CCR1 IL10 IL12A TNF TNFRSF1A
43 11.21 IL10 IL12A LTA TNF
44
Show member pathways
11.18 IL10 IL12A IL23R LTA STAT4 TLR4
45 11.16 NOD2 TNF TNFRSF1A
46
Show member pathways
10.91 FAS IL10 IL12A TNF TNFRSF1A

GO Terms for Behcet Syndrome

Cellular components related to Behcet Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.81 ADA2 C4A ERAP1 FAS IL10 IL12A
2 extracellular space GO:0005615 9.56 ADA2 C4A ERAP1 IL10 IL12A LTA
3 cell surface GO:0009986 9.1 FAS HLA-B NOD2 TLR4 TNF TNFRSF1A

Biological processes related to Behcet Syndrome according to GeneCards Suite gene sharing:

(show all 47)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.93 CCR1 NOD2 TLR4 TNF
2 immune system process GO:0002376 9.92 C4A ERAP1 HLA-B IL23R MEFV NOD2
3 positive regulation of I-kappaB kinase/NF-kappaB signaling GO:0043123 9.9 NOD2 TLR4 TNF TNFRSF1A
4 defense response to Gram-positive bacterium GO:0050830 9.88 IL12A LTA TNF
5 negative regulation of inflammatory response GO:0050728 9.87 IL10 MEFV TNFRSF1A
6 cellular response to mechanical stimulus GO:0071260 9.85 FAS TLR4 TNFRSF1A
7 apoptotic signaling pathway GO:0097190 9.85 FAS TLR4 TNF
8 regulation of inflammatory response GO:0050727 9.85 NOD2 TLR4 TNF
9 tumor necrosis factor-mediated signaling pathway GO:0033209 9.85 FAS LTA TNF TNFRSF1A
10 defense response to bacterium GO:0042742 9.85 IL10 NOD2 TLR4 TNF TNFRSF1A
11 positive regulation of JNK cascade GO:0046330 9.84 NOD2 TLR4 TNF
12 cellular response to lipopolysaccharide GO:0071222 9.84 IL10 IL12A TLR4 TNF
13 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.83 IL12A IL23R TNFRSF1A
14 I-kappaB kinase/NF-kappaB signaling GO:0007249 9.8 TLR4 TNF TNFRSF1A
15 positive regulation of interleukin-6 production GO:0032755 9.77 NOD2 TLR4 TNF
16 extrinsic apoptotic signaling pathway GO:0097191 9.75 FAS IL12A TNF
17 positive regulation of nitric-oxide synthase biosynthetic process GO:0051770 9.7 NOD2 TLR4
18 response to lipopolysaccharide GO:0032496 9.7 FAS IL10 IL12A IL23R LTA TLR4
19 positive regulation of interleukin-17 production GO:0032740 9.69 IL23R NOD2
20 negative regulation of interleukin-12 production GO:0032695 9.69 IL10 MEFV
21 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.69 IL10 TNF
22 positive regulation of interleukin-8 production GO:0032757 9.69 NOD2 TLR4 TNF
23 positive regulation of T cell mediated cytotoxicity GO:0001916 9.68 IL12A IL23R
24 interferon-gamma production GO:0032609 9.68 IL12A TLR4
25 negative regulation of interleukin-17 production GO:0032700 9.68 IL12A TLR4
26 negative regulation of interleukin-6 production GO:0032715 9.67 IL10 TLR4 TNF
27 interleukin-12-mediated signaling pathway GO:0035722 9.67 IL10 IL12A IL23R STAT4
28 positive regulation of ceramide biosynthetic process GO:2000304 9.66 TNF TNFRSF1A
29 positive regulation of interleukin-8 biosynthetic process GO:0045416 9.65 TLR4 TNF
30 positive regulation of lymphocyte proliferation GO:0050671 9.65 IL12A TLR4
31 positive regulation of interferon-gamma production GO:0032729 9.65 IL12A IL23R LTA TLR4 TNF
32 death-inducing signaling complex assembly GO:0071550 9.64 TNF TNFRSF1A
33 endothelial cell apoptotic process GO:0072577 9.64 IL10 TNF
34 regulation of establishment of endothelial barrier GO:1903140 9.62 TNF TNFRSF1A
35 positive regulation of MHC class II biosynthetic process GO:0045348 9.62 IL10 TLR4
36 necroptotic signaling pathway GO:0097527 9.61 FAS TNF
37 negative regulation of cytokine secretion involved in immune response GO:0002740 9.61 IL10 TNF
38 positive regulation of osteoclast differentiation GO:0045672 9.61 CCR1 IL23R TNF
39 immune response GO:0006955 9.61 CCR1 FAS HLA-B IL10 IL12A LTA
40 positive regulation of NK T cell activation GO:0051135 9.58 IL12A IL23R
41 negative regulation of growth of symbiont in host GO:0044130 9.58 IL10 LTA TNF
42 positive regulation of humoral immune response mediated by circulating immunoglobulin GO:0002925 9.57 LTA TNF
43 receptor biosynthetic process GO:0032800 9.52 IL10 TNF
44 positive regulation of chronic inflammatory response to antigenic stimulus GO:0002876 9.48 LTA TNF
45 inflammatory response GO:0006954 9.32 C4A CCR1 FAS IL10 IL23R MEFV
46 regulation of receptor activity GO:0010469 10.06 ADA2 IL10 IL12A LTA TNF
47 innate immune response GO:0045087 10.03 C4A IL23R MEFV NOD2 PSTPIP1 TLR4

Molecular functions related to Behcet Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.46 IL10 IL12A LTA TNF
2 interleukin-12 receptor binding GO:0005143 8.96 IL12A IL23R
3 tumor necrosis factor binding GO:0043120 8.62 FAS TNFRSF1A

Sources for Behcet Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....