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Behcet Syndrome (CUI C0004943) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0004943

NCI Thesaurus Code: C34416  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: A rare chronic inflammatory disorder of unknown etiology. It is characterized by the development of ulcers in the mouth and genital region and uveitis. Other signs and symptoms include arthritis, deep vein thrombosis and superficial thrombophlebitis.

GARD Definition: Behcet's 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 Behcet's disease is still unknown.   Treatment is symptomatic and supportive. Experience is evolving with the use of interferon-alpha and with agents which inhibit tumor necrosis factor (TNF) in the treatment of Behçet’s disease. Behcet's disease is a lifelong disorder that comes and goes. Spontaneous remission over time is common for individuals with Behçet’s disease but permanent remission of symptoms has not been reported. - this information is from GARD/ORDR/NCATS.

MEDLINEPLUS Definition: 

Behcet's syndrome is a disease that involves 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

NICHD Definition: A systemic vasculitis affecting both arteries and veins which manifests primarily as recurrent oral and genital ulceration, uveitis, characteristic rash, and arthritis. It may also cause central nervous system disease, gastrointestinal inflammation or ulceration, or thrombophlebitis.

CSP Definition: rare chronic inflammatory disease involving the small blood vessels; of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region and uveitis with hypopyon; the neuroocular form may cause blindness and death; synovitis, thrombophlebitis, gastrointestinal ulcerations, retinal vasculitis, and optic atrophy may occur as well.

MSH Definition: 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.

Synonyms & Abbreviations: (see Synonym Details)
Adamantiades-Behcet Disease
Adamantiades-Behcet Diseases
ADAMANTIADES-BEHCET SYNDROME
Adamantiades Behcet Disease
BD
Behcet's
Behcet's disease
Behcet's syndrome
Behcet's syndrome (disorder)
Behcets disease
Behcets Syndrome
BEHCET DIS
Behcet disease
BEHCET RECURRENT DISEASE
BEHCET SYNDROME
Behcet Syndrome [Disease/Finding]
BEHCET TRIPLE SYMPTOM COMPLEX
Behçet disease
Behçet's disease
Behçet's Syndrome
Behçet-Adamantiades Syndrome
Behçet Disease
Behçet Diseases
Behçet Syndrome
Complex, Triple Symptom
Complices, Triple Symptom
Disease, Adamantiades-Behcet
Disease, Behçet
Diseases, Adamantiades-Behcet
Diseases, Behçet
Morbus Behçet's Syndrome
OCULOBUCCOGENITAL SYNDROME
Old Silk Route Disease
Silk Road Disease
Symptom Complex, Triple
Symptom Complices, Triple
Triple-Symptom Complex
triple symptom complex
Triple Symptom Complices

External Source Codes: 
NCI Thesaurus Code C34416 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AN in translations spell Behçet's, not Behcet's MSH
AQL BL CF CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
Contributing_Source NICHD NCI
CTV3ID AD61. SNOMEDCT_US
DATE_CREATED 01/05/2000 MEDLINEPLUS
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2015-01-22T00:04:00 GARD
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 0944-7825 CSP
DISEASE_IDENTIFIER_ID 30418 GARD
DISEASE_IDENTIFIER_ID 7378 GARD
DISEASE_IDENTIFIER_ID 7379 GARD
DISEASE_IDENTIFIER_ID 7380 GARD
DISEASE_IDENTIFIER_ID 7381 GARD
DX 20030101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
EXTERNALLY_DEFINED N LNC
HAS_GARD_PAGE true GARD
HN 2003 (1963) MSH
IAN DEFAULT ICD10
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_DATE 2010-02-24T00:00:00 GARD
MDA 19990101 MSH
MESH_DEFINITION 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. NDFRT
MESH_DUI D001528 NDFRT
MESH_NAME Behcet Syndrome NDFRT
MESH_UI M0002296 NDFRT
MIMTYPE 5 OMIM
MIMTYPEMEANING Mendelian phenotype or locus, molecular basis unknown. OMIM
MIMTYPEVALUE perc OMIM
MMR 20150622 MSH
MN C07.465.075 MSH
MN C11.941.879.780.880.200 MSH
MN C14.907.940.100 MSH
MN C16.320.382.250 MSH
MN C17.800.827.368.250 MSH
MN C17.800.862.150 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
MODULE_ID 731000124108 SNOMEDCT_US
MODULE_NAME US National Library of Medicine maintained module SNOMEDCT_US
MP_HEALTH_TOPIC_URL https://www.nlm.nih.gov/medlineplus/behcetssyndrome.html MEDLINEPLUS
MP_OTHER_LANGUAGE_URL Spanish https://www.nlm.nih.gov/medlineplus/spanish/behcetssyndrome.html MEDLINEPLUS
MP_PRIMARY_INSTITUTE_URL National Institute of Arthritis and Musculoskeletal and Skin Diseases http://www.niams.nih.gov/ MEDLINEPLUS
NDFRT_KIND DISEASE_KIND NDFRT
NICHD_Hierarchy_Term Behcet Syndrome NCI
NUI N0000000539 NDFRT
ORDER_NO 17996 ICD10CM
PM 2003; see BEHCET'S SYNDROME 1963-2002 MSH
PRIMARY_PATH 10004213$10047113$10047116$10047065$Behcet's syndrome$Vasculitides NEC$Vascular inflammations$Vascular disorders MDR
PRIMARY_SOC 10047065 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/848/behcets-disease/resources/1 GARD
RXAUI 3116814 RXNORM
RXAUI 3116815 RXNORM
RXAUI 3116816 RXNORM
RXAUI 3116817 RXNORM
RXAUI 3250887 RXNORM
RXAUI 5930140 RXNORM
RXAUI 5930361 RXNORM
RXAUI 5930362 RXNORM
RXAUI 5931836 RXNORM
RXAUI 5932384 RXNORM
RXCUI 985793 RXNORM
SMQ_TERM_ADDVERSION 10.1 MDR
SMQ_TERM_ADDVERSION 12.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 12.0 MDR
SMQ_TERM_LMVERSION 13.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 310701003 NDFRT
SNOMEDID DF-0024C SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS M35.2 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~M35.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS M35.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~M35.2 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~AD61. SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~DF-0024C SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T004437 MSH
TERMUI T004438 MSH
TERMUI T459777 MSH
TERMUI T459778 MSH
TERMUI T840990 MSH
TERMUI T840991 MSH
TERMUI T840992 MSH
TERMUI T840993 MSH
TERMUI T840994 MSH
TH GHR (2014) MSH
TH NLM (1966) MSH
TH NLM (2003) MSH
TH NLM (2014) MSH
TH OMIM (2013) MSH
TH ORD (2010) MSH
TH UNK (19XX) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0004943

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