Behçet disease

Información en español Title

Other Names:

Behçet's syndrome; Behçet syndrome; BD; Behçet's syndrome; Behçet syndrome; BD; Behcet syndrome; Behcet's disease; Behçet’s disease; Behcet's syndrome; Behçet's disease See More

Categories:

Blood Diseases; Eye diseases; Kidney and Urinary Diseases; Blood Diseases; Eye diseases; Kidney and Urinary Diseases; Musculoskeletal Diseases; Nervous System Diseases; Skin Diseases See More

Summary Summary

Behçet disease leads to swelling of the blood vessels and affects multiple organs throughout the body. Symptoms generally begin when individuals are in their 20s or 30s, but may occur at any age .Symptoms may include ulcers affecting the mouth and genitals, various skin lesions, and swelling of the membranes affecting the eyes. In some people, symptoms include arthritis (swollen, painful, stiff joints), skin problems, and inflammation of the digestive tract, brain, and spinal cord. Symptoms of Behçet disease may be active and then become inactive, but they never completely go away. In some cases, Behçet disease may lead to vision loss and neurological problems. The exact cause of Behçet disease is unknown, but it is thought to be due to a combination of genetic, immune, and environmental factors. Diagnosis is based on the symptoms and clinical examination. Diagnostic guidelines have been published. Treatment is focused on managing the symptoms and includes steroids and drugs that suppress the immune system.^[1]^[2]^[3]^[4]

Last updated: 3/31/2021

Symptoms Symptoms

The following list includes the most common signs and symptoms in people with Behçet disease. These features may be different from person to person. Some people may have more symptoms than others and they can range from mild to severe. This list does not include every symptom that has been described in the condition.

Signs and symptoms may include:^[3]^[4]

Ulcers (sores) inside the mouth or genitals
Inflammation of the eye
Eye pain
Blurred vision
Skin lesions
Joint pain
Ulcers in the digestive tract
Inflammation of the brain or spinal cord which can result in neurological damage
Inflammation of the blood vessels

The symptoms of Behçet disease usually begin in the 20s or 30s, although they may happen at any age. Symptoms may come and go in episodes, but do not usually go away. In severe cases, vision problems and neurological damage may occur over time.^[3]

Last updated: 3/31/2021

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Arthritis	Joint inflammation	0001369
Fatigue	Tired Tiredness [ more ]	0012378
Fever		0001945
Meningitis		0001287
Migraine	Intermittent migraine headaches Migraine headache Migraine headaches [ more ]	0002076
Myalgia	Muscle ache Muscle pain [ more ]	0003326
Nausea and vomiting		0002017
Oral ulcer	Mouth ulcer	0000155
Orchitis	Inflammation of testicles	0100796
Papule		0200034
Photophobia	Extreme sensitivity of the eyes to light Light hypersensitivity [ more ]	0000613
Recurrent aphthous stomatitis	Recurrent canker sores	0011107
Subcutaneous nodule	Firm lump under the skin Growth of abnormal tissue under the skin [ more ]	0001482
Vasculitis	Inflammation of blood vessel	0002633
30%-79% of people have these symptoms
Abdominal pain	Pain in stomach Stomach pain [ more ]	0002027
Abnormal blistering of the skin	Blistering, generalized Blisters [ more ]	0008066
Acne		0001061
Arthralgia	Joint pain	0002829
Confusion	Disorientation Easily confused Mental disorientation [ more ]	0001289
Gait disturbance	Abnormal gait Abnormal walk Impaired gait [ more ]	0001288
Gastrointestinal hemorrhage	Gastrointestinal bleeding	0002239
Hemiparesis	Weakness of one side of body	0001269
Immunologic hypersensitivity		0100326
Venous thrombosis	Blood clot in vein	0004936
5%-29% of people have these symptoms
Abnormal myocardium morphology		0001637
Abnormal pyramidal sign		0007256
Anorexia		0002039
Aortic regurgitation		0001659
Arterial thrombosis	Blood clot in artery	0004420
Ataxia		0001251
Avascular necrosis	Death of bone due to decreased blood supply	0010885
Blindness		0000618
Cataract	Clouding of the lens of the eye Cloudy lens [ more ]	0000518
Cerebral ischemia	Disruption of blood oxygen supply to brain	0002637
Cranial nerve paralysis		0006824
Developmental regression	Loss of developmental milestones Mental deterioration in childhood [ more ]	0002376
Encephalitis	Brain inflammation	0002383
Endocarditis		0100584
Gangrene	Death of body tissue due to lack of blood flow or infection	0100758
Glomerulopathy		0100820
Hemoptysis	Coughing up blood	0002105
Hyperreflexia	Increased reflexes	0001347
Increased intracranial pressure	Rise in pressure inside skull	0002516
Irritability	Irritable	0000737
Keratoconjunctivitis sicca	Dry eyes	0001097
Lymphadenopathy	Swollen lymph nodes	0002716
Malabsorption	Intestinal malabsorption	0002024
Memory impairment	Forgetfulness Memory loss Memory problems Poor memory [ more ]	0002354
Mitral regurgitation		0001653
Myocardial infarction	Heart attack	0001658
Myositis	Muscle inflammation	0100614
Pancreatitis	Pancreatic inflammation	0001733
Paresthesia	Pins and needles feeling Tingling [ more ]	0003401
Pericarditis	Swelling or irritation of membrane around heart	0001701
Pleural effusion	Fluid around lungs	0002202
Pleuritis	Inflammation of tissues lining lungs and chest	0002102
Pulmonary embolism	Blood clot in artery of lung	0002204
Pulmonary infiltrates	Lung infiltrates	0002113
Renal insufficiency	Renal failure Renal failure in adulthood [ more ]	0000083
Retinopathy	Noninflammatory retina disease	0000488
Retrobulbar optic neuritis		0100654
Seizure		0001250
Splenomegaly	Increased spleen size	0001744
Vertigo	Dizzy spell	0002321
Weight loss		0001824
Percent of people who have these symptoms is not available through HPO
Autosomal recessive inheritance		0000007
Chorioretinitis		0012424
Decreased level of D-mannose in urine		0410060
Epididymitis		0000031
Erythema		0010783
Erythema nodosum		0012219
Genital ulcers		0003249
Hypopyon		0031615
Iridocyclitis		0001094
Iritis	Inflammation of iris	0001101
Patchy alopecia	Patchy baldness	0002232
Raynaud phenomenon		0030880
Superficial thrombophlebitis		0002638

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 2/1/2021

Cause Cause

The exact cause of Behçet disease is unknown. Genetic, environmental factors, and abnormalities of the immune system may be involved.^[1]

Last updated: 3/31/2021

Diagnosis Diagnosis

Behcet disease is diagnosed based on the symptoms and clinical examination. Blood and urine tests may be done as well. Guidelines for the diagnosis of Behcet disease have been published.^[3]^[4]^[5]

Last updated: 3/31/2021

Treatment Treatment

Treatment of Behçet disease is focused on managing the symptoms. Options for treatment includes steroids, drugs that suppress the immune system, drugs that block inflammation, and medications that are put on the skin.

The European League Against Rheumatism (EULAR) has recommendations for the management of Behçet disease.^[6]

Last updated: 3/31/2021

Statistics Statistics

The number of people with Behcet disease is different in different parts of the world. Behcet disease is more common in the Middle East and Mediterranean regions than other parts of the world. In these regions, it has been estimated that between 20-420 people per 100,000 has Behcet disease.^[3] The exact number of people with this condition in the US is unknown.

Last updated: 3/31/2021

Do you have updated information on this disease? We want to hear from you.

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from MedlinePlus Genetics.
The Vasculitis Foundation has a "Find a Physician" map on its website.

Related Diseases Related Diseases

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Depending upon manifestations, infectious uveitis, relapsing polychondritis, sarcoidosis, antiphospholipid syndrome, Takayasu arteritis, Crohn disease or multiple sclerosis (see these terms) may be considered. Visit the Orphanet disease page for more information.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
ClinicalTrials.gov lists trials that are related to Behçet disease. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Patient Registry

The Autoimmune Registry supports research for Behçet disease by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.
The Vasculitis Clinical Research Consortium (VCRC) is an integrated group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research in different forms of vasculitis. The VCRC has a contact registry for patients who wish to be contacted about clinical research opportunities and updates on the progress of the VCRC research projects.

For more information on the registry see: https://www.rarediseasesnetwork.org/cms/vcrc/About-Us

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

American Behcet's Disease Association (ABDA)
PO Box 80576
Rochester, MN 48308
Toll-free: 1-800-723-4238 or 1-800-7-BEHCET (Hotline)
Telephone: +1-631-656-0537
E-mail: info@behcets.com
Website: https://www.behcets.com/
Behcet's Organisation Worldwide
Head Office
PO Box 27
Watchet
Somerset
TA23 0YJ
United Kingdom
E-mail: http://www.behcets.org/4728.html
Website: http://www.behcets.org/
FMF & AID Global Association
Schüracherstrasse 25a
8306 Brüttisellen
Switzerland
Telephone: +41 76 415 4010
E-mail: info@fmfandaid.org
Website: https://www.fmfandaid.org/
Vasculitis Foundation
P.O. Box 28660
Kansas City, MO 64188
Toll-free: 1-800-277-9474
Telephone: +1-816-436-8211
Fax: +1-816-656-3838
E-mail: https://www.vasculitisfoundation.org/contact-us-2/
Website: https://www.vasculitisfoundation.org/

Social Networking Websites

RareConnect has an online community for patients and families with this condition so they can connect with others and share their experiences living with a rare disease. The project is a joint collaboration between EURORDIS (European Rare Disease Organisation) and NORD (National Organization for Rare Disorders).

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The Johns Hopkins Vasculitis Center website provides information about different types of vasculitis. Click on the link to access information about Behcet's disease.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
Genetics Home Reference (GHR) contains information on Behçet disease. This website is maintained by the National Library of Medicine.
The National Eye Institute (NEI) was established by Congress in 1968 to protect and prolong the vision of the American people. Click on the link to view information on this topic.
The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases, the training of basic and clinical scientists to carry out this research, and the dissemination of information on research progress in these diseases. Click on the link to view information on this topic.
The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Behçet disease. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References

Smith EL, Yazici Y. Pathogenesis of Behçet syndrome. UpToDate. updated Aug. 26, 2020; https://www.uptodate.com/contents/pathogenesis-of-behcet-syndrome.
Bulur I, Onder M. Behçet disease: New aspects. Clin Dermatol. Sep-Oct, 2017; 35(5):421-434. https://pubmed.ncbi.nlm.nih.gov/28916023/.
Davatchi F, Chams-Davatchi C, Shams H, Shahram F, Nadji A, Akhlaghi M, et al. Behcet's disease: epidemiology, clinical manifestations, and diagnosis. Expert Rev Clin Immunol. Jan 2017; 13(1):57-65. https://pubmed.ncbi.nlm.nih.gov/27351485/.
Greco A, De Virgilio A, Ralli M, Ciofalo A, Mancini P, Attanasio G, et al. Behçet's disease: New insights into pathophysiology, clinical features and treatment options.. Autoimmun Rev. Jun 2018; 17(6):567-575. https://pubmed.ncbi.nlm.nih.gov/29631062/.
Davatchi F, Abdollahi BS, Chams-Davatchi C, Shahram F, Nadji A, Shams H, et al. Validation of the revised International Criteria for Behcet's Disease (ICBD) in Iran.. Clin Rheumatol. Feb 2015; 34(2):315-20. https://pubmed.ncbi.nlm.nih.gov/24150745/.
Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis. Jun 2018; 77(6):808-818. https://pubmed.ncbi.nlm.nih.gov/29625968/.
Questions and Answers about Behçet’s Disease. National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). 2015; http://www.niams.nih.gov/Health_Info/Behcets_Disease/default.asp.
Bashour M. Ophthalmologic Manifestations of Behcet Disease Treatment & Management. Medscape Reference. 2016; http://emedicine.medscape.com/article/1229174-treatment.