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Behçet’s disease

Behçet's Disease is a chronic rheumatic illness, initiated through the bodies immune system being over-reactive, and producing unpredictable episodes of inflammation which affect mostly the small blood vessels. Symptoms may occur wherever there is a blood supply

Prevelance

1-9 / 100 000

3,310 - 29,790

US Estimated

5,135 - 46,215

Europe Estimated

Age of Onset

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ICD-10

M35.2

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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Rare View

Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful mouth sores, genital sores, inflammation of parts of the eye, and arthritis. The syndrome is rare in the United States, Africa and South America, but is common in the Middle East and Asia.

5 Facts you should know

FACT

1

 Behçet disease is a rare vasculitic disorder that is characterized by the triad of recurrent oral aphthous ulcers, genital ulcers, and uveitis

FACT

2

Behçet’s disease is virtually unparalleled among the vasculitides in its ability to involve blood vessels of nearly all sizes and types, ranging from small arteries to large ones

FACT

3

The disease is named after a Turkish dermatologist, Hulusi Behçet, who noted the disease triad and became the first physician to describe the disease in modern times

FACT

4

Symptoms generally begin when individuals are in their 20s or 30s, but may occur at any age

FACT

5

Behçet’s disease is most common along the “Old Silk Route,” which spans the region from Japan and China in the Far East to the Mediterranean Sea, including countries such as Turkey and Iran

Behçet's disease is also know as...

Behçet's disease

Behçet syndrome; BD

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Common signs & symptoms

Sores inside the mouth or genitals

Eye problems

Pus-filled lesions and other problems on the skin

Pain in the joints

Recurring ulcers in the digestive tract

Problems of the central nervous system

Inflammation of the blood vessels

Vasculitis

Current treatments

Although there is no cure for Behçet disease, people can usually control symptoms with proper medication, rest, exercise, and a healthy lifestyle. The goal of treatment is to reduce discomfort and prevent serious complications such as disability from arthritis or blindness. The type of medicine and the length of treatment depend on the person's symptoms and their severity. It is likely that a combination of treatments will be needed to relieve specific symptoms. Patients should tell each of their doctors about all of the medicines they are taking so that the doctors can coordinate treatment.[3]

Topical medicine is applied directly on the sores to relieve pain and discomfort. For example, doctors prescribe rinses, gels, or ointments. Creams are used to treat skin and genital sores. The medicine usually contains corticosteroids (which reduce inflammation), other anti-inflammatory drugs, or an anesthetic, which relieves pain.[3]

Doctors also prescribe medicines taken by mouth to reduce inflammation throughout the body, suppress the overactive immune system, and relieve symptoms. Doctors may prescribe one or more of the medicines listed below to treat the various symptoms of Behçet disease.[3]

Interferon-alfa, azathioprine, and TNF-α blockers may be tried in rare cases of patients with resistant, prolonged, and disabling attacks.[5]

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

For ocular disease, azathioprine is the first medication that should be used. For severe eye disease (such as drop in visual acuity, retinal vasculitis, or macular involvement), either cyclosporine A or infliximab may be used in combination with azathioprine and corticosteroids. Interferon-alfa, alone or in combination with corticosteroids, appears to be a second choice in this eye disease.[6]

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsLocationsMore Information
Topical Pentoxifylline Gel on Behcet's Disease Genital UlcersBehçet's Disease (BD) is a complex, chronic, relapsing, multi-system inflammatory disorder that is characterized by oral ulcers, genital ulcers in addition to ocular and skin symptoms. Genital ulcers are the second most common symptom of BD and occur in 57-93% of BD patients. The genital ulcers of BD clearly cause considerable morbidity for those affected. This will be one of the first trials of a topical product designed for accelerated healing of BD genital ulcers. There is no cure or FDA-approved therapies for BD genital ulcers and the treatment of recurrent genital ulcers that are associated with BD is palliative.

Recent study evaluating the effect of topical PTX on BD oral ulcers suggests that topical pentoxifylline (PTX) might have an immediate impact on BD oral ulcer healing leading to accelerated ulcer clearance, which results in lower daily ulcer numbers for the patients along with reduced pain scores. Investigators hypothesize that application of topical PTX will accelerate the healing of BD genital ulcers in a similar clinically meaningful way, and further hypothesize that topical PTX can become a valuable adjunct to any other systemic therapy for BD.
Phase 2Not yet recruitingDrug: Topical Pentoxifylline Gel (Vehicle +PTX)|Drug: Topical Placebo Gel (Vehicle)Silk Road Therapies, Washington, District of Columbia, United StatesMore Information
A Phase 3 Randomized, Double-blind Study to Evaluate the Efficacy and Safety of Apremilast (CC-10004) in Subjects With Active Behçet's DiseaseThis study will evaluate if apremilast is better than placebo (inactive substance in the same form as the drug) for the treatment of oral ulcers in subjects with active Behçet's disease. Other manifestations of the disease will also be assessed, such as, pain and tenderness in joints, eye inflammation, genital ulcers, and skin disease. This study also will test how well the body tolerates apremilast. In addition, the second purpose of the study is to assess the safety of apremilast in patients with Behçet's disease.

This study is a randomized, placebo-controlled, parallel design. The placebo-controlled period will be 12 weeks long and participants will receive apremilast or placebo. After the 12-week placebo-controlled period, all participants will receive apremilast for 52 weeks in the active treatment period. All participants will have their final study visit 4 weeks after stopping apremilast treatment.
Phase 3CompletedDrug: Apremilast|Drug: PlaceboArizona Arthritis and Rheumatology Research, PLLC, Phoenix, Arizona, United States|University of California Davis Medical Center, Sacramento, California, United States|Millennium Research, Ormond Beach, Florida, United States|Arthritis and Rheumatology of Georgia, Atlanta, Georgia, United States|Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States|Advanced Rheumatology, Lansing, Michigan, United States|Shores Rheumatology, Saint Clair Shores, Michigan, United States|University of New Mexico, Albuquerque, New Mexico, United States|New York Methodist Hospital, Brooklyn, New York, United States|NYU Langone Medical Center, New York, New York, United States|University of Pennsylvania Health Systems, Philadelphia, Pennsylvania, United States|Hopital de La Conception, Marseille Cedex 05, France|Pitié-Salpêtriere Hospital Paris, Paris, France|Hopital Cochin, Paris, France|Hospital Saint Louis, Paris, France|Charite Universitaetsmedizin Berlin, Berlin, Germany|Stadtisches Klinikum Dessau, Dessau, Germany|Asklepios Rheumazentrum Hamburg, Hamburg, Germany|Navy Hospital of Athens, Athens, Greece|Athens General Hospital 'G Gennimatas', Athens, Greece|Laiko General Hospital of Athens, Athens, Greece|Ippokratio General Hospital of Thessaloniki, Thessaloniki, Greece|Bnai Zion Medical Center, Haifa, Israel|Rambam Health Care Campus, Haifa, Israel|Hadassah Medical Organization, Jerusalem, Israel|Rabin Medical Center, Petah Tikva, Israel|Chaim Sheba Medical Center, Ramat Gan, Israel|Azienda Ospedaliero Universitaria Careggi, Firenze, Italy|Azienda Ospedaliera Regionale San Carlo, Potenza/Matera, Italy|Arcispedale Santa Maria Nuova, Reggio Emilia, Italy|Medical Hospital of Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan|Nippon Medical School Hospital, Bunkyo-ku, Japan|St. Luke's International Hospital, Chuo-ku, Japan|Tokyo Metropolitan Tama Medical Center, Fuchu-shi, Japan|Japanese Red Cross Society Himeji Hospital, Himeji-shi, Japan|Saitama Medical University Hospital, Iruma-gun, Saitama, Japan|Teikyo University Hospital, Itabashi-ku, Japan|Nihon University Itabashi Hospital, Itabashi-ku, Japan|St Marianna University School of Medicine Hospital, Kawasaki, Kanagawa, Japan|University of Occupational and Environmentall Health, Kitakyushu, Japan|Kagawa University Hospital, Miki-cho, Japan|Kitasato University Hospital, Sagamihara, Japan|Saga Medical School Hospital, Saga, Japan|Sapporo Medical University Hospital, Sapporo, Hokkaidô, Japan|Hokkaido University Hospital, Sapporo, Hokkaidô, Japan|Shimonoseki City Hospital, Shimonoseki, Japan|Tokyo Medical University Hospital, Shinjyuku-ku, Japan|Tomishiro Central Hospital, Tomigusuku-shi, Japan|Ehime University Hospital, Toon, Japan|Yokohama City University Hospital, Yokohama, Kanagawa, Japan|Chungnam National University Hospital, Daejeon, Korea, Republic of|Seoul National University Hospital, Seoul, Korea, Republic of|Severance Hospital, Yonsei University Health System, Seoul, Korea, Republic of|Konkuk University Hospital, Seoul, Korea, Republic of|Ajou University Hospital, Suwon si, Korea, Republic of|Hotel Dieu de France, Beirut, Lebanon|Ain Wazein Hospital, El Chouf, Lebanon|American University of Beirut Medical Center, El Chouf, Lebanon|Cukurova University Medical Faculty Balcali Hospital, Adana, Turkey|Eskisehir Osmangazi University, Eskisehir, Turkey|Istanbul Universitesi Cerrahpasa Tip Fakultesi, Istanbul, Turkey|Marmara University Hospital, Istanbul, Turkey|Selcuk University Medical Faculty, Konya, TurkeyMore Information
Safety and Efficacy of AIN457 in Noninfectious UveitisThis study was performed to evaluate the efficacy and safety of AIN457 for patients with active uveitis that requires systemic immunosuppression.Phase 2CompletedDrug: AIN457|Drug: AIN 457Novartis Investigative Site, Beverly Hills, California, United States|Novartis Investigative Site, Los Angeles, California, United States|Novartis Investigative Site, Sacramento, California, United States|Novartis Investigative Site, Denver, Colorado, United States|Novartis Investigative Site, Golden, Colorado, United States|Novartis Investigative Site, Littleton, Colorado, United States|Novartis Investigative Site, Atlanta, Georgia, United States|Novartis Investigative Site, Baltimore, Maryland, United States|Novartis Investigative Site, Baltimore, Maryland, United States|Novartis Investigative Site, Cambridge, Massachusetts, United States|Novartis Investigative Site, Kansas City, Missouri, United States|Novartis Investigative Site, Teaneck, New Jersey, United States|Novartis Investigative Site, New York, New York, United States|Novartis Investigative Site, Slingerlands, New York, United States|Novartis Investigative Site, Durham, North Carolina, United States|Novartis Investigative Site, Cleveland, Ohio, United States|Novartis Investigative Site, Spartanburg, South Carolina, United States|Novartis Investigative Site, Arlington, Texas, United States|Novartis Investigative Site, Austin, Texas, United States|Novartis Investigative Site, Houston, Texas, United States|Novartis Investigative Site, Berlin, Germany|Novartis Investigative Site, Heidelberg, Germany|Novartis Investigative Site, Tübingen, GermanyMore Information

Top Treatments in Research

AgentClass/Mechanism of ActionDevelopment StatusCompanyCompany ContactClinical StudiesMore Information
Topical Pentoxifylline GelPentoxifylline belongs to a class of drugs known as hemorrheologic agents. It works by helping blood flow more easily through narrowed arteries. This increases the amount of oxygen that can be delivered by the blood when the muscles need more (such as during exercise) thereby increasing walking distance and duration.Phase 2Silk Road Therapies
Washington, District of Columbia, United States, 20008
Timothy Cote, MD 202-738-6336 timcote1960@gmail.com Click here for more information

National Institute of Arthritis and Musculoskeletal and Skin Diseases