MCID: BHC003
MIFTS: 62

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 54 12 50 25 42 38 69 50
Behcet's Disease 38 12 50 51 14
Behcet's Syndrome 12 50 25 41
Behçet Disease 50 25 56
Adamantiades-Behcet Disease 12 25
Triple Symptom Complex 12 25
Behcet Disease 25 52
Behcet Triple Symptom Complex 25
Old Silk Route Disease 25
Malignant Aphthosis 25
Behçet's Syndrome 50
Behet's Syndrome 12
Behçet's Disease 50
Behçet’s Disease 50
Bd 50

Characteristics:

Orphanet epidemiological data:

56
behçet disease
Inheritance: Multigenic/multifactorial; Prevalence: 1-5/10000 (Japan),>1/1000 (Turkey); Age of onset: Adolescent,Adult,Childhood; Age of death: adult;

OMIM:

54
Inheritance:
familial cases reported, but probably not mendelian


Classifications:



Summaries for Behcet Syndrome

NINDS : 51 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 bd syndrome and ptosis coloboma mental retardation, and has symptoms including ataxia, acne and hyperreflexia. 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 PEDF Induced Signaling. The drugs Fluocinolone Acetonide and Methylprednisolone 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 hematopoietic system

NIH Rare Diseases : 50 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 : 41 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)
id Related Disease Score Top Affiliating Genes
1 bd syndrome 11.9
2 ptosis coloboma mental retardation 11.3 MEFV NOD2 PSTPIP1
3 acute neonatal citrullinemia type i 11.3 MEFV TNFRSF1A
4 acute megakaryoblastic leukemia without down syndrome 11.3 MEFV TNFRSF1A
5 congenital insensitivity to pain with hyperhidrosis 11.3 IL10 NOD2 TLR4
6 pure autonomic failure 11.3 IL10 TNF
7 mechanical lagophthalmos 11.3 MEFV TNF TNFRSF1A
8 multiple sclerosis 5 11.3 MEFV TNF TNFRSF1A
9 lip cancer 11.2 MEFV NOD2 PSTPIP1 TNF
10 noma 11.2 HLA-B IL10 MEFV TNF
11 myopathy 11.2 MEFV NOD2 PSTPIP1 TNF
12 yao syndrome 11.2 MEFV NOD2 TNF
13 cyclosporiasis 11.2 HLA-B IL10 TNF
14 familial mediterranean fever, ad 11.2 MEFV PSTPIP1 TNF TNFRSF1A
15 hemophagocytic lymphohistiocytosis 11.2 HLA-B IL10 TNF
16 type 1 papillary adenoma of the kidney 11.2 IL10 LTA TNF
17 cryptococcosis 11.2 HLA-B IL10 TNF
18 antigen-peptide-transporter 2 deficiency 11.2 HLA-B TLR4 TNF
19 acute monoblastic leukemia 11.2 IL10 NOD2 TNF
20 liver leiomyoma 11.2 IL10 LTA TNF
21 psoriasis susceptibility 1 11.2 HLA-B NOD2 TNF TNFRSF1A
22 alkhurma hemorrhagic fever 11.2 IL10 TLR4 TNF
23 esophagus melanoma 11.2 IL10 MEFV TLR4 TNF
24 dermatomycosis 11.2 C4A MEFV TNF
25 high pressure neurological syndrome 11.2 HLA-B IL10 TNF
26 patau syndrome 11.2 MEFV TNF TNFRSF1A
27 acute ackee fruit intoxication 11.2 IL10 TNF
28 aseptic meningitis 11.2 HLA-B TLR4 TNF
29 esophageal candidiasis 11.2 HLA-B IL10 NOD2 TNF
30 primary biliary cirrhosis 11.2 IL10 LTA TNF TNFRSF1A
31 megaloblastic anemia 11.2 IL10 TLR4 TNF
32 opportunistic bacterial infectious disease 11.2 IL10 MEFV TLR4 TNF
33 benign eccrine breast spiradenoma 11.2 IL10 NOD2 TLR4 TNF
34 atypical lichen myxedematosus 11.2 FAS STAT4 TNF
35 spondylocostal dysostosis 4 11.2 LTA TLR4 TNF
36 bartonellosis 11.2 IL10 TLR4 TNF
37 gastroduodenal crohn's disease 11.2 NOD2 TLR4 TNF
38 orbital osteomyelitis 11.2 HLA-B IL10 TNF
39 idiopathic dilatation of the pulmonary artery 11.2 IL10 IL23R LTA TNF
40 disease of mental health 11.2 HLA-B IL10 TNF
41 autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome 11.2 FAS IL10 TNF
42 tuberculum sellae meningioma 11.2 IL10 NOD2 TLR4 TNF
43 pancreas lymphoma 11.2 MEFV TNF TNFRSF1A
44 membranoproliferative glomerulonephritis 11.2 FAS NOD2 TNF
45 palmoplantar keratosis 11.2 IL10 TLR4 TNF
46 cornea cancer 11.2 HLA-B IL10 TNF
47 aspartylglucosaminuria 11.2 IL10 LTA NOD2 TNF
48 abnormal retinal correspondence 11.2 IL10 TLR4 TNF
49 iida kannari syndrome 11.2 IL10 TLR4 TNF
50 melioidosis 11.2 IL10 TLR4 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:

54

Joints:
arthritis

Eyes:
iritis
uveitis
iridocyclitis
hypopyon
choreoretinitis

Hair:
alopecia areata

Mouth:
mouth ulcerations

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

Neuro:
schizoaffective disorder
brainstem syndrome
meningoencephalomyelitic syndrome
organic confusional state

GU:
epididymitis
genital ulcerations


Clinical features from OMIM:

109650

Human phenotypes related to Behcet Syndrome:

56 32 (show top 50) (show all 76)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 ataxia 56 32 occasional (7.5%) Occasional (29-5%) HP:0001251
2 acne 56 32 frequent (33%) Frequent (79-30%) HP:0001061
3 hyperreflexia 56 32 occasional (7.5%) Occasional (29-5%) HP:0001347
4 migraine 56 32 hallmark (90%) Very frequent (99-80%) HP:0002076
5 splenomegaly 56 32 occasional (7.5%) Occasional (29-5%) HP:0001744
6 seizures 56 32 occasional (7.5%) Occasional (29-5%) HP:0001250
7 irritability 56 32 occasional (7.5%) Occasional (29-5%) HP:0000737
8 fatigue 56 32 hallmark (90%) Very frequent (99-80%) HP:0012378
9 renal insufficiency 56 32 occasional (7.5%) Occasional (29-5%) HP:0000083
10 pancreatitis 56 32 occasional (7.5%) Occasional (29-5%) HP:0001733
11 blindness 56 32 occasional (7.5%) Occasional (29-5%) HP:0000618
12 cataract 56 32 occasional (7.5%) Occasional (29-5%) HP:0000518
13 photophobia 56 32 hallmark (90%) Very frequent (99-80%) HP:0000613
14 encephalitis 56 32 occasional (7.5%) Occasional (29-5%) HP:0002383
15 pericarditis 56 32 occasional (7.5%) Occasional (29-5%) HP:0001701
16 pleuritis 56 32 occasional (7.5%) Occasional (29-5%) HP:0002102
17 arthritis 56 32 hallmark (90%) Very frequent (99-80%) HP:0001369
18 myalgia 56 32 hallmark (90%) Very frequent (99-80%) HP:0003326
19 memory impairment 56 32 occasional (7.5%) Occasional (29-5%) HP:0002354
20 hemiparesis 56 32 frequent (33%) Frequent (79-30%) HP:0001269
21 confusion 56 32 frequent (33%) Frequent (79-30%) HP:0001289
22 fever 56 32 hallmark (90%) Very frequent (99-80%) HP:0001945
23 malabsorption 56 32 occasional (7.5%) Occasional (29-5%) HP:0002024
24 abdominal pain 56 32 frequent (33%) Frequent (79-30%) HP:0002027
25 venous thrombosis 56 32 frequent (33%) Frequent (79-30%) HP:0004936
26 lymphadenopathy 56 32 occasional (7.5%) Occasional (29-5%) HP:0002716
27 pulmonary embolism 56 32 occasional (7.5%) Occasional (29-5%) HP:0002204
28 vertigo 56 32 occasional (7.5%) Occasional (29-5%) HP:0002321
29 weight loss 56 32 occasional (7.5%) Occasional (29-5%) HP:0001824
30 developmental regression 56 32 occasional (7.5%) Occasional (29-5%) HP:0002376
31 vasculitis 56 32 hallmark (90%) Very frequent (99-80%) HP:0002633
32 hemoptysis 56 32 occasional (7.5%) Occasional (29-5%) HP:0002105
33 myocardial infarction 56 32 occasional (7.5%) Occasional (29-5%) HP:0001658
34 retinopathy 56 32 occasional (7.5%) Occasional (29-5%) HP:0000488
35 gangrene 56 32 occasional (7.5%) Occasional (29-5%) HP:0100758
36 arthralgia 56 32 frequent (33%) Frequent (79-30%) HP:0002829
37 gait disturbance 56 32 frequent (33%) Frequent (79-30%) HP:0001288
38 arterial thrombosis 56 32 occasional (7.5%) Occasional (29-5%) HP:0004420
39 aortic regurgitation 56 32 occasional (7.5%) Occasional (29-5%) HP:0001659
40 pleural effusion 56 32 occasional (7.5%) Occasional (29-5%) HP:0002202
41 pulmonary infiltrates 56 32 occasional (7.5%) Occasional (29-5%) HP:0002113
42 mitral regurgitation 56 32 occasional (7.5%) Occasional (29-5%) HP:0001653
43 recurrent aphthous stomatitis 56 32 hallmark (90%) Very frequent (99-80%) HP:0011107
44 anorexia 56 32 occasional (7.5%) Occasional (29-5%) HP:0002039
45 cranial nerve paralysis 56 32 occasional (7.5%) Occasional (29-5%) HP:0006824
46 cerebral ischemia 56 32 occasional (7.5%) Occasional (29-5%) HP:0002637
47 meningitis 56 32 hallmark (90%) Very frequent (99-80%) HP:0001287
48 increased intracranial pressure 56 32 occasional (7.5%) Occasional (29-5%) HP:0002516
49 paresthesia 56 32 occasional (7.5%) Occasional (29-5%) HP:0003401
50 keratoconjunctivitis sicca 56 32 occasional (7.5%) Occasional (29-5%) HP:0001097

UMLS symptoms related to Behcet Syndrome:


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

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

26
id 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 ADA2 C4A
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 TLR4 CCR1 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 IL10 C4A
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 ADA2 C4A HLA-B 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 CCR1 C4A
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 ADA2 IL10
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
51 Increased shRNA abundance (Z-score > 2) GR00366-A-93 10.21 ADA2
52 Increased shRNA abundance (Z-score > 2) GR00366-A-97 10.21 ADA2

MGI Mouse Phenotypes related to Behcet Syndrome:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.25 LTA MEFV NOD2 PSTPIP1 STAT4 TLR4
2 immune system MP:0005387 10.17 CCR1 ERAP1 FAS IL10 IL12A IL23R
3 homeostasis/metabolism MP:0005376 10.15 MEFV NOD2 STAT4 TLR4 TNF TNFRSF1A
4 digestive/alimentary MP:0005381 10.03 CCR1 FAS IL10 IL23R NOD2 TLR4
5 liver/biliary system MP:0005370 9.86 TNFRSF1A CCR1 FAS IL10 LTA MEFV
6 neoplasm MP:0002006 9.7 FAS IL10 IL12A IL23R TLR4 TNF
7 respiratory system MP:0005388 9.56 CCR1 FAS IL10 IL12A LTA TLR4
8 skeleton MP:0005390 9.28 FAS IL10 IL12A LTA MEFV NOD2

Drugs & Therapeutics for Behcet Syndrome

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

(show top 50) (show all 82)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fluocinolone Acetonide Approved, Investigational, Vet_approved Phase 4 67-73-2 6215
2
Methylprednisolone Approved, Vet_approved Phase 4,Phase 3,Phase 2 83-43-2 6741
3
Prednisolone Approved, Vet_approved Phase 4,Phase 3,Phase 2 50-24-8 5755
4 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2
5 glucocorticoids Phase 4
6 Hormone Antagonists Phase 4
7 Hormones Phase 4
8 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
9 Antiemetics Phase 4
10 Antineoplastic Agents, Hormonal Phase 4
11 Autonomic Agents Phase 4
12 Gastrointestinal Agents Phase 4,Phase 3,Phase 1,Phase 2
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 Antifungal Agents Phase 3
28 Anti-Infective Agents Phase 3,Phase 2
29 Antirheumatic Agents Phase 3,Phase 2,Phase 1
30 Antiviral Agents Phase 3,Phase 2
31 Calcineurin Inhibitors Phase 3
32 Cyclosporins Phase 3
33 Dermatologic Agents Phase 3,Phase 1,Phase 2
34 Immunosuppressive Agents Phase 3,Phase 2
35 Interferon-alpha Phase 3,Phase 2
36 interferons Phase 3,Phase 2
37 Analgesics Phase 3,Phase 2
38 Analgesics, Non-Narcotic Phase 3,Phase 2
39 Anti-Inflammatory Agents, Non-Steroidal Phase 3,Phase 2
40 Antibodies Phase 3,Phase 2
41 Antibodies, Monoclonal Phase 3,Phase 2
42 Immunoglobulins Phase 3,Phase 2
43 Angiogenesis Inhibitors Phase 3,Phase 2
44 Angiogenesis Modulating Agents Phase 3,Phase 2
45 Anti-Bacterial Agents Phase 3,Phase 2
46
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
47
Colchicine Approved Phase 2 64-86-8 6167 2833
48
Azathioprine Approved Phase 2 446-86-6 2265
49
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
50
rituximab Approved Phase 2 174722-31-7 10201696

Interventional clinical trials:

(show top 50) (show all 61)

id Name Status NCT ID Phase Drugs
1 Flucinolone Acetonide Implant for Treating Refractory Ocular Behcet's Disease Unknown status NCT00720928 Phase 4 flucinolone acetonide
2 The Efficacy of Methylprednisolone in the Treatment of Patients With Ocular Involvement in Behcet's Disease Unknown status NCT01306955 Phase 4 methylorednisolone
3 Saline vs. Lactated Ringers for Emergency Department IV Fluid Resuscitation Recruiting 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 A Study of Adalimumab in Japanese Subjects With Intestinal Behçet's Disease Completed NCT01243671 Phase 3
7 Clinical Study of TA-650 in Patients With Behcet's Disease (BD) With Special Lesions Completed NCT01532570 Phase 3 TA-650
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 A Study to Evaluate Efficacy and Safety of Infliximab in Participant With Moderate-to-Severe Refractory Intestinal Behcet's Disease Recruiting NCT02505568 Phase 3 Infliximab
11 Interferon α2a Versus Cyclosporine for Refractory Behçet`s Disease Uveitis Recruiting NCT03209219 Phase 3 Interferon Alfa-2A;Cyclosporine Pill
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 Thalidomide Versus Infliximab in New Onset Crohn's Disease With Poor Prognostic Factors Not yet recruiting NCT03221166 Phase 3 Thalidomide;Infliximab
14 Efficacy and Safety Study of Gevokizumab to Treat Behcet's Disease Uveitis Terminated NCT02258867 Phase 3 Placebo;Gevokizumab
15 Efficacy of Gevokizumab in the Treatment of Patients With Behçet's Disease Uveitis (EYEGUARD™-B) Terminated NCT01965145 Phase 3 Gevokizumab;Placebo
16 Long-term Safety of Gevokizumab in the Treatment of Patients With Chronic Non-infectious Uveitis (EYEGUARD-X) Terminated NCT02375685 Phase 3
17 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
18 Colchicine Randomized Double-Blind Controlled Crossover Study in Behcet's Disease Completed NCT00700297 Phase 2 Colchicine;Placebo
19 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
20 Rituximab for the Treatment of Severe Ocular Manifestations of Behcet's Disease Completed NCT00664599 Phase 2 Rituximab;Cytotoxic Combination
21 Low Dose Interferon Alpha Treatment for Oral Ulcers of Behcet's Disease Completed NCT00483184 Phase 2
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 HAT in Eye Complications of Behcet's Disease Completed NCT00001865 Phase 2 Daclizumab
24 Anakinra for Behcet s Disease Completed NCT01441076 Phase 1, Phase 2 Anakinra
25 Ilaris (Canakinumab) in the Schnitzler Syndrome Completed NCT01245127 Phase 2 Ilaris
26 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
27 1 Year Canakinumab Treatment in Behcet's Disease Patients With Neurologic or Vascular Involvement Recruiting NCT02756650 Phase 2 drug administration
28 Induction of Regulatory t Cells by Low Dose il2 in Autoimmune and Inflammatory Diseases Recruiting NCT01988506 Phase 2 Interleukin 2
29 Efficacy and Safety of Ustekinumab, a Human Monoclonal Anti-IL-12/IL-23 Antibody, in Patients With Behçet Disease Not yet recruiting NCT02648581 Phase 2 Subcutaneous Ustekinumab
30 Tocilizumab for the Treatment of Behcet's Syndrome Terminated NCT01693653 Phase 2 Tocilizumab
31 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
32 Stem Cell Transplantation for the Treatment of Knee Osteoarthritis Unknown status NCT00550524 Phase 1
33 Stem Cell Transplantation in Ocular Lesions of Behcet's Disease Terminated NCT00550498 Phase 1
34 Hematopoietic Stem Cell Support in Vasculitis Terminated NCT00278512 Phase 1
35 Etanercept: Single Blind Control Study in Ocular Manifestations of Behcet's Disease Unknown status NCT00931957 Etanercept, Methotrexate, Prednisolone
36 Psychological Symptoms in Patients With Behcet's Disease by SCL90-R Completed NCT00699985
37 Special Investigation in Patients With Intestinal Behcet's Disease (All Case Investigation) Completed NCT01960790
38 Behçet's Disease and Eosinophil Cationic Protein Completed NCT01584778
39 MEVALONATE KINASE GENE MUTATIONS AND THEIR CLINICAL CORRELATIONS IN BEHÇET'S DISEASE Completed NCT01780363
40 The Relationship Between Serum Levels of Angiogenin, bFGF, VEGF and Ocular Involvement in Patients With Behçet's Disease Completed NCT01720628
41 Biological Markers in Retinal Vasculitis Completed NCT00050492
42 Educational Needs of Patients With Systemic Vasculitis Completed NCT02190929
43 Uveitis Gene-Expression Profiling Completed NCT00874471
44 Impact of Vasculitis on Employment and Income Completed NCT02476292
45 Vasculitis Illness Perception (VIP) Study Completed NCT02190916
46 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
47 Steroid-Induced Osteoporosis in the Pediatric Population - Canadian Incidence Study Completed NCT01663129
48 Evaluation and Treatment of Patients With Inflammatory Eye Diseases Completed NCT00006188
49 A Prospective, Mono-Country and Multi-center Study to Observe Safety and Effectiveness of Adalimumab in KoREan Intestinal Behcet's Disease(BD) Patients Recruiting NCT02687828
50 Immunogenetic Mechanisms in Behcet's Disease Recruiting NCT01109433

Search NIH Clinical Center for Behcet Syndrome

Inferred drug relations via UMLS 69 / NDF-RT 48 :


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:

39
Eye, Skin, Brain, Spinal Cord, Bone, Neutrophil, T Cells

Publications for Behcet Syndrome

Articles related to Behcet Syndrome:

id 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 13)
id 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.927G> A (p.Met309Ile) single nucleotide variant Pathogenic rs146597836 GRCh38 Chromosome 22, 17189987: 17189987
9 ADA2 NM_001282225.1(ADA2): c.740C> T (p.Ala247Val) single nucleotide variant Pathogenic rs750868279 GRCh38 Chromosome 22, 17203576: 17203576
10 MEFV NM_000243.2(MEFV): c.1211A> G (p.His404Arg) single nucleotide variant Pathogenic rs755659290 GRCh38 Chromosome 16, 3249480: 3249480
11 MEFV NM_000243.2(MEFV): c.1099C> G (p.Leu367Val) single nucleotide variant Pathogenic rs1057519328 GRCh38 Chromosome 16, 3249592: 3249592
12 ADA2 NM_001282225.1(ADA2): c.1045G> A (p.Val349Ile) single nucleotide variant Pathogenic rs74317375 GRCh37 Chromosome 22, 17669265: 17669265
13 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
id 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 43)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.93 ADA2 C4A CCR1 ERAP1 HLA-B IL10
2
Show member pathways
13.66 CCR1 FAS IL10 IL12A IL23R LTA
3
Show member pathways
13.36 CCR1 FAS IL10 IL12A IL23R LTA
4
Show member pathways
13.35 CCR1 HLA-B IL10 IL12A IL23R LTA
5
Show member pathways
13.3 CCR1 FAS IL10 IL12A IL23R LTA
6
Show member pathways
12.88 FAS IL10 IL12A TLR4 TNF TNFRSF1A
7
Show member pathways
12.78 FAS HLA-B IL12A LTA TLR4 TNF
8
Show member pathways
12.63 FAS IL12A LTA TLR4 TNF TNFRSF1A
9
Show member pathways
12.6 C4A FAS HLA-B IL10 IL12A KLRC4
10 12.54 FAS IL10 STAT4 TNF TNFRSF1A
11
Show member pathways
12.51 IL10 IL12A IL23R NOD2 STAT4 TLR4
12
Show member pathways
12.4 FAS IL10 PSTPIP1 TNF
13 12.39 HLA-B LTA TNF TNFRSF1A
14
Show member pathways
12.26 IL10 IL12A TLR4 TNF TNFRSF1A
15
Show member pathways
12.2 IL10 IL12A IL23R STAT4
16
Show member pathways
12.13 HLA-B IL10 IL12A LTA TNF
17 12.13 IL10 IL12A NOD2 TLR4 TNF TNFRSF1A
18 11.99 IL10 IL12A IL23R TNF
19 11.93 FAS LTA NOD2 TNF TNFRSF1A
20 11.9 IL10 IL12A TLR4 TNF
21 11.9 CCR1 IL10 IL12A IL23R STAT4
22 11.89 LTA TLR4 TNF TNFRSF1A
23
Show member pathways
11.87 IL23R STAT4 TLR4
24
Show member pathways
11.83 FAS LTA TNF TNFRSF1A
25 11.74 IL10 IL12A TNF
26 11.72 IL10 LTA TLR4 TNF
27 11.71 TLR4 TNF TNFRSF1A
28 11.67 IL10 IL12A IL23R LTA TNF
29 11.6 LTA TLR4 TNF TNFRSF1A
30 11.6 C4A IL10 IL12A TLR4 TNF
31 11.59 IL12A TLR4 TNF
32 11.55 FAS TNF TNFRSF1A
33
Show member pathways
11.53 LTA TNF TNFRSF1A
34 11.53 IL10 IL12A TLR4 TNF
35 11.51 IL12A TLR4 TNF
36 11.47 FAS TNF TNFRSF1A
37 11.36 IL10 TLR4 TNF
38 11.32 FAS IL10 IL12A TNF
39 11.24 CCR1 IL10 IL12A TNF TNFRSF1A
40 11.21 IL10 IL12A LTA TNF
41
Show member pathways
11.2 IL10 IL12A IL23R LTA STAT4 TLR4
42 11.14 NOD2 TNF TNFRSF1A
43
Show member pathways
10.96 FAS IL10 IL12A TNF TNFRSF1A

GO Terms for Behcet Syndrome

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

id Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.81 ADA2 C4A ERAP1 FAS IL10 IL12A
2 external side of plasma membrane GO:0009897 9.56 CCR1 FAS TLR4 TNF
3 extracellular space GO:0005615 9.56 ADA2 C4A ERAP1 IL10 IL12A LTA
4 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 45)
id 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 9.87 IL12A LTA TNF
5 negative regulation of inflammatory response GO:0050728 9.87 IL10 MEFV TNFRSF1A
6 cellular response to lipopolysaccharide GO:0071222 9.86 IL10 IL12A TLR4 TNF
7 cellular response to mechanical stimulus GO:0071260 9.85 FAS TLR4 TNFRSF1A
8 apoptotic signaling pathway GO:0097190 9.85 FAS TLR4 TNF
9 tumor necrosis factor-mediated signaling pathway GO:0033209 9.85 FAS LTA TNF TNFRSF1A
10 positive regulation of JNK cascade GO:0046330 9.84 NOD2 TLR4 TNF
11 response to glucocorticoid GO:0051384 9.84 FAS IL10 TNF
12 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.83 IL12A IL23R TNFRSF1A
13 defense response to bacterium GO:0042742 9.83 IL10 NOD2 TLR4 TNF 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 response to lipopolysaccharide GO:0032496 9.7 FAS IL10 IL12A IL23R LTA TLR4
18 positive regulation of nitric-oxide synthase biosynthetic process GO:0051770 9.69 NOD2 TLR4
19 positive regulation of interleukin-17 production GO:0032740 9.69 IL23R NOD2
20 extrinsic apoptotic signaling pathway via death domain receptors GO:0008625 9.69 FAS TNF TNFRSF1A
21 negative regulation of interleukin-12 production GO:0032695 9.68 IL10 MEFV
22 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.68 IL10 TNF
23 positive regulation of T cell mediated cytotoxicity GO:0001916 9.68 IL12A IL23R
24 negative regulation of interleukin-17 production GO:0032700 9.67 IL12A TLR4
25 positive regulation of interleukin-8 production GO:0032757 9.67 NOD2 TLR4 TNF
26 positive regulation of ceramide biosynthetic process GO:2000304 9.66 TNF TNFRSF1A
27 positive regulation of lymphocyte proliferation GO:0050671 9.65 IL12A TLR4
28 positive regulation of interleukin-8 biosynthetic process GO:0045416 9.65 TLR4 TNF
29 negative regulation of interleukin-6 production GO:0032715 9.65 IL10 TLR4 TNF
30 positive regulation of interferon-gamma production GO:0032729 9.65 IL12A IL23R LTA TLR4 TNF
31 death-inducing signaling complex assembly GO:0071550 9.64 TNF TNFRSF1A
32 endothelial cell apoptotic process GO:0072577 9.64 IL10 TNF
33 positive regulation of MHC class II biosynthetic process GO:0045348 9.63 IL10 TLR4
34 regulation of establishment of endothelial barrier GO:1903140 9.62 TNF TNFRSF1A
35 necroptotic signaling pathway GO:0097527 9.61 FAS TNF
36 negative regulation of cytokine secretion involved in immune response GO:0002740 9.61 IL10 TNF
37 immune response GO:0006955 9.61 CCR1 FAS HLA-B IL10 IL12A LTA
38 positive regulation of NK T cell activation GO:0051135 9.58 IL12A IL23R
39 positive regulation of osteoclast differentiation GO:0045672 9.58 CCR1 IL23R TNF
40 positive regulation of humoral immune response mediated by circulating immunoglobulin GO:0002925 9.57 LTA TNF
41 negative regulation of growth of symbiont in host GO:0044130 9.54 IL10 LTA TNF
42 receptor biosynthetic process GO:0032800 9.52 IL10 TNF
43 positive regulation of chronic inflammatory response to antigenic stimulus GO:0002876 9.46 LTA TNF
44 inflammatory response GO:0006954 9.32 C4A CCR1 FAS IL10 IL23R MEFV
45 innate immune response GO:0045087 10.01 C4A IL23R MEFV NOD2 PSTPIP1 TLR4

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

id 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
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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