BehÇet's disease is an inflammatory disorder of unknowncause, characterized by recurrent oral aphthous ulcers, genitalulcers, uveitis, and skin lesions.1,2 All these common manifestationsare self-limiting except for the ocular attacks. Repeated attacksof uveitis can cause blindness.1,2,3 Behçet's diseaseis not a chronic, persistent inflammatory disease, but ratherone consisting of recurrent attacks of acute inflammation. Involvementof the gastrointestinal tract, central nervous system, and largevessels is less frequent (Table 1), although it can be life-threatening.1,2,4,5,6Susceptibility to Behçet's disease is strongly associatedwith the presence of the HLA-B51 allele.7,8 Environmental factorssuch as infectious . . . [Full Text of this Article]
Epidemiology
Causation
Pathophysiology
Diagnosis
Clinical Manifestations
Treatment
Mucocutaneous Lesions
Ocular Lesions
Arthritis
Gastrointestinal Lesions
Central Nervous System Lesions
Large-Vessel Involvement
Conclusions
Source Information
From the Departments of Immunology and Medicine, St. Marianna University School of Medicine, Kawasaki (T.S., M.T., N.S.); and the Department of Ophthalmology, Uveitis Clinic, Tokyo Women's Medical College and Daini Hospital, Tokyo (G.I.) all in Japan.
Address reprint requests to Dr. Sakane at the Departments of Immunology and Medicine, St. Marianna University School of Medicine, 2-16-1, Sugao, Miyamae-ku, Kawasaki, Kanagawa, 216-8511, Japan, or at t2sakane@marianna-u.ac.jp.
References
Related Letters:
Behçet's Disease
Agca M., Nzerue C. M., Ehrlich G. E., Drenth J. P.H., Livneh A., Haznedaroglu I. C., Özcebe O. I., Dündar S. V., Cheng T. O., Sakane T., Takeno M., Suzuki N.
Extract |
Full Text
N Engl J Med 2000;
342:587-589, Feb 24, 2000.
Correspondence
This article has been cited by other articles:
Kappen, J. H., Wallace, G. R., Stolk, L., Rivadeneira, F., Uitterlinden, A. G., van Daele, P. L. A., Laman, J. D., Kuijpers, R. W. A. M., Baarsma, G. S., Stanford, M. R., Fortune, F., Madanat, W., van Hagen, P. M., van Laar, J. A. M.
(2009). Low prevalence of NOD2 SNPs in Behcet's disease suggests protective association in Caucasians. Rheumatology (Oxford)
48: 1375-1377
[Abstract][Full Text]
Hsin, I-F., Tsai, C.-Y.
(2009). Association between sarcoidosis and Behcet disease in a young woman with symptoms mimicking myasthaenia gravis. BMJ Case Reports
2009: bcr0920080874-bcr0920080874
[Abstract][Full Text]
Jeong, D. S., Kim, K.-H., Kim, J. S., Ahn, H.
(2009). Long-Term Experience of Surgical Treatment for Aortic Regurgitation Attributable to Behcet's Disease.. Ann. Thorac. Surg.
87: 1775-1782
[Abstract][Full Text]
Takahama, M., Yamamoto, R., Nakajima, R., Tada, H.
(2009). Successful surgical treatment of pulmonary artery aneurysm in Behcet's syndrome. ICVTS
8: 390-392
[Abstract][Full Text]
Kawakami, T., Yamazaki, M., Mizoguchi, M., Soma, Y.
(2009). Antiphosphatidylserine-Prothrombin Complex Antibodies in 3 Patients With Behcet Disease Involving Superficial Vein Thrombophlebitis. Arch Dermatol
145: 171-175
[Abstract][Full Text]
Priya, C. G., Rathinam, S. R., Muthukkaruppan, V.
(2008). Evidence for Endotoxin as a Causative Factor for Leptospiral Uveitis in Humans. IOVS
49: 5419-5424
[Abstract][Full Text]
Atkinson, M., Moore, E., Altinok, D., Acsadi, G.
(2008). Cerebral Infarct in Pediatric Neuro-Behcet's Disease. J Child Neurol
23: 1331-1335
[Abstract]
Ahn, J. K., Cha, H.-S., Koh, E.-M., Kim, S.-H., Kim, Y. G., Lee, C.-K., Yoo, B.
(2008). Behcet's disease associated with bone marrow failure in Korean patients: clinical characteristics and the association of intestinal ulceration and trisomy 8. Rheumatology (Oxford)
47: 1228-1230
[Abstract][Full Text]
Kuemmerle-Deschner, J. B., Tzaribachev, N., Deuter, C., Zierhut, M., Batra, M., Koetter, I.
(2008). Interferon-{alpha}--a new therapeutic option in refractory juvenile Behcet's disease with CNS involvement. Rheumatology (Oxford)
47: 1051-1053
[Abstract][Full Text]
Gornik, H. L., Creager, M. A.
(2008). Aortitis. Circulation
117: 3039-3051
[Full Text]
Sheehan, N. J.
(2008). Dysphagia and other manifestations of oesophageal involvement in the musculoskeletal diseases. Rheumatology (Oxford)
47: 746-752
[Abstract][Full Text]
Meguro, A, Ota, M, Katsuyama, Y, Oka, A, Ohno, S, Inoko, H, Mizuki, N
(2008). Association of the toll-like receptor 4 gene polymorphisms with Behcet's disease. Ann Rheum Dis
67: 725-727
[Full Text]
Sanudo-Buitrago, F, Gonzalez-Meijome, J M, Bueno-Jimeno, I, Navarrete-Sanchis, J N, Leon-Jimenez, N
(2008). Topographic and refractive changes in a patient with contact lens intolerance associated with Behcet disease. Br J Ophthalmol
92: 438-440
[Full Text]
Kitaichi, N., Miyazaki, A., Iwata, D., Ohno, S., Stanford, M. R, Chams, H.
(2007). Ocular features of Behcet's disease: An international collaborative study. Br J Ophthalmol
91: 1579-1582
[Abstract][Full Text]
Umehara, N., Saito, S., Ishii, H., Aomi, S., Kurosawa, H.
(2007). Rupture of Thoracoabdominal Aortic Aneurysm Associated with Behcet's Disease. Ann. Thorac. Surg.
84: 1394-1396
[Abstract][Full Text]
Atzeni, F., Boiardi, L., Casali, B., Farnetti, E., Sarzi-Puttini, P., Pipitone, N., Olivieri, I., Cantini, F., Salvi, F., La Corte, R., Triolo, G., Filippini, D., Paolazzi, G., Salvarani, C.
(2007). Lack of association of the 463 G/A myeloperoxidase promoter polymorphism with Behcet's disease in Italian patients. Rheumatology (Oxford)
46: 1547-1550
[Abstract][Full Text]
Knockaert, D. C.
(2007). Cardiac involvement in systemic inflammatory diseases. Eur Heart J
28: 1797-1804
[Abstract][Full Text]
Niccoli, L., Nannini, C., Benucci, M., Chindamo, D., Cassara, E., Salvarani, C., Cimino, L., Gini, G., Lenzetti, I., Cantini, F.
(2007). Long-term efficacy of infliximab in refractory posterior uveitis of Behcet's disease: a 24-month follow-up study. Rheumatology (Oxford)
46: 1161-1164
[Abstract][Full Text]
Sfikakis, P. P., Markomichelakis, N., Alpsoy, E., Assaad-Khalil, S., Bodaghi, B., Gul, A., Ohno, S., Pipitone, N., Schirmer, M., Stanford, M., Wechsler, B., Zouboulis, C., Kaklamanis, P., Yazici, H.
(2007). Anti-TNF therapy in the management of Behcet's disease--review and basis for recommendations. Rheumatology (Oxford)
46: 736-741
[Full Text]
McDonald, D. R., Lee, C., Fowler, R. A., Abuhaleeqa, K.
(2007). Behcet's disease. CMAJ
176: 1273-1274
[Full Text]
van Laar, J A M, Missotten, T, van Daele, P L A, Jamnitski, A, Baarsma, G S, van Hagen, P M
(2007). Adalimumab: a new modality for Behcet's disease?. Ann Rheum Dis
66: 565-566
[Full Text]
Nakao, K., Isashiki, Y., Sonoda, S., Uchino, E., Shimonagano, Y., Sakamoto, T.
(2007). Nitric Oxide Synthase and Superoxide Dismutase Gene Polymorphisms in Behcet Disease. Arch Ophthalmol
125: 246-251
[Abstract][Full Text]
Akar, S., Ozcan, M. A., Ates, H., Gurler, O., Alacacioglu, I., Ozsan, G. H., Akkoc, N., Ozkan, S., Demirkan, F., Onen, F.
(2006). Circulated Activated Platelets and Increased Platelet Reactivity in Patients With Behcet's Disease. CLIN APPL THROMB HEMOST
12: 451-457
[Abstract]
Suzuki, N., Nara, K., Suzuki, T.
(2006). Skewed Th1 Responses Caused by Excessive Expression of Txk, a Member of the Tec Family of Tyrosine Kinases, in Patients with Behcet's Disease.. Clin Med Res
4: 147-151
[Abstract][Full Text]
Unger, P., de Canniere, D., Preumont, N., Fesler, P., Cogan, E.
(2006). Subaortic obstruction and complete atrioventricular block in Behcet's disease. Eur J Echocardiogr
7: 250-252
[Abstract][Full Text]
Mat, C., Yurdakul, S., Uysal, S., Gogus, F., Ozyazgan, Y., Uysal, O., Fresko, I., Yazici, H.
(2006). A double-blind trial of depot corticosteroids in Behcet's syndrome. Rheumatology (Oxford)
45: 348-352
[Abstract][Full Text]
Calguneri, M., Aydemir, K., Ozturk, M. A., Haznedaroglu, I. C., Kiraz, S., Ertenli, I.
(2006). Myocardial Infarction and Deep Venous Thrombosis in a Young Patient with Behcet Disease. CLIN APPL THROMB HEMOST
12: 105-109
[Abstract]
Maurer, B, Hensel, M, Max, R, Fiehn, C, Ho, A D, Lorenz, H M
(2006). Autologous haematopoietic stem cell transplantation for Behcet's disease with pulmonary involvement: analysis after 5 years of follow up. Ann Rheum Dis
65: 127-129
[Abstract][Full Text]
Uzun, O., Akpolat, T., Erkan, L.
(2005). Pulmonary Vasculitis in Behcet Disease: A Cumulative Analysis. Chest
127: 2243-2253
[Abstract][Full Text]
Hisanaga, K., Iwasaki, Y., Itoyama, Y., the Neuro-Sweet Disease Study Group,
(2005). Neuro-Sweet disease: Clinical manifestations and criteria for diagnosis. Neurology
64: 1756-1761
[Abstract][Full Text]
Kathiresan, S., Kelsey, P. B., Steere, A. C., Foster, C. S., Curvelo, M. S., Stone, J. R.
(2005). Case 14-2005 - A 38-Year-Old Man with Fever and Blurred Vision. NEJM
352: 2003-2012
[Full Text]
Karasneh, J. A., Hajeer, A. H., Silman, A., Worthington, J., Ollier, W. E. R., Gul, A.
(2005). Polymorphisms in the endothelial nitric oxide synthase gene are associated with Behcet's disease. Rheumatology (Oxford)
44: 614-617
[Abstract][Full Text]
Sonoda, K.-H., Inaba, S., Ariyama, A., Kawano, Y.-I., Saniabadi, A., Ishibashi, T.
(2005). Therapeutic Neutrophil Apheresis in Patients With Ocular Behcet Disease. Arch Ophthalmol
123: 267-269
[Full Text]
Bayazit, Y A, Evereklioglu, C, Ozer, E, Kirtak, N, Bayazit, N, Kanlikama, M, Inaloz, S
(2004). Neurotological status in Behcet's disease and its ophthalmological correlates. Postgrad. Med. J.
80: 724-728
[Abstract][Full Text]
Leiba, M, Seligsohn, U, Sidi, Y, Harats, D, Sela, B A, Griffin, J H, Livneh, A, Rosenberg, N, Gelernter, I, Gur, H, Ehrenfeld, M
(2004). Thrombophilic factors are not the leading cause of thrombosis in Behcet's disease. Ann Rheum Dis
63: 1445-1449
[Abstract][Full Text]
Mirsattari, S. M., McGinn, G. J., Halliday, W. C.
(2004). Neuro-Behcet disease with predominant involvement of the brainstem. Neurology
63: 382-384
[Abstract][Full Text]
Hama, Y, Kaji, T, Iwasaki, Y, Kawauchi, T, Yamamoto, M, Kusano, S
(2004). Endovascular management of multiple arterial aneurysms in Behcet's disease. Br. J. Radiol.
77: 615-619
[Abstract][Full Text]
Beckman, J. A, Pfizenmaier, D. H II, Rooke, T. W
(2004). Clinical pathologic conference: A young man with a thoracoabdominal aneurysm. Vasc Med
9: 70-77
Rossi, G., Moretta, A., Locatelli, F.
(2004). Autologous hematopoietic stem cell transplantation for severe/refractory intestinal Behcet disease. Blood
103: 748-750
[Full Text]
Chang, H K, Kim, J U, Lee, S S, Yoo, D H
(2004). Lack of association between angiotensin converting enzyme gene polymorphism and Korean Behcet's disease. Ann Rheum Dis
63: 106-107
[Full Text]
Lockwood, C. M., Hale, G., Waldman, H., Jayne, D. R. W.
(2003). Remission induction in Behcet's disease following lymphocyte depletion by the anti-CD52 antibody CAMPATH 1-H. Rheumatology (Oxford)
42: 1539-1544
[Abstract][Full Text]
Kim, J U, Chang, H K, Lee, S S, Kim, J W, Kim, K T, Lee, S W, Chung, W T
(2003). Endothelial nitric oxide synthase gene polymorphisms in Behcet's disease and rheumatic diseases with vasculitis. Ann Rheum Dis
62: 1083-1087
[Abstract][Full Text]
Oztuirk, M. A., Ozbalkan, Z., Onat, A. M., Ertenli, I., Kiraz, S., Aytemir, K., Ureten, K., Abali, G., Calguineri, M., Kirazli, S., Haznedaroglu, I. C.
(2003). Decreased Protein Z Concentrations Complicating the Hypercoagulable State of Behqet's Disease. CLIN APPL THROMB HEMOST
9: 259-263
[Abstract]
Karasneh, J., Hajeer, A. H., Barrett, J., Ollier, W. E. R., Thornhill, M., Gul, A.
(2003). Association of specific interleukin 1 gene cluster polymorphisms with increased susceptibility for Behcet's disease. Rheumatology (Oxford)
42: 860-864
[Abstract][Full Text]
Sigirci, A., Senol, M., Aydin, E., Kutlu, R., Alkan, A., Altinok, M. T., Yologlu, S., Baysal, T., Sarac, K.
(2003). Doppler Waveforms and Blood Flow Parameters of the Superior and Inferior Mesenteric Arteries in Patients Having Behcet Disease With and Without Gastrointestinal Symptoms: Preliminary Data. J Ultrasound Med
22: 449-457
[Abstract][Full Text]
Suzuki, N, Takeno, M, Inaba, G
(2003). Bilateral subdural effusion in a patient with neuro-Behcet's disease. Ann Rheum Dis
62: 374-375
[Full Text]
Taysi, S., Kocer, I., Memisogullari, R., Kiziltunc, A.
(2002). Serum Oxidant/Antioxidant Status in Patients with Behcet's Disease. Annals of Clinical & Laboratory Science
32: 377-382
[Abstract][Full Text]
Morcos, Z
(2002). Recurrent orogenital ulcers with papilloedema and headaches. Postgrad. Med. J.
78: 317-317
[Full Text]
Leiba, M, Sidi, Y, Gur, H, Leiba, A, Ehrenfeld, M
(2001). Behcet's disease and thrombophilia. Ann Rheum Dis
60: 1081-1085
[Full Text]
Direskeneli, H
(2001). Behcet's disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum Dis
60: 996-1002
[Full Text]
Kari, J. A., Shah, V., Dillon, M. J.
(2001). Behcet's disease in UK children: clinical features and treatment including thalidomide. Rheumatology (Oxford)
40: 933-938
[Abstract][Full Text]
Planer, D, Verstandig, A, Chajek-Shaul, T
(2001). Transcatheter embolization of renal artery aneurysm in Behcet's disease. Vasc Med
6: 109-112
[Abstract]
Pamuk, G. E., Pamuk, O. N., Tabak, F., Mert, A., Ozturk, R., Aktuglu, Y.
(2001). Systemic Nocardia infection in a patient with Behcet's disease. Rheumatology (Oxford)
40: 597-599
[Full Text]
Chambers, J. C., Haskard, D. O., Kooner, J. S.
(2001). Vascular endothelial function and oxidative stress mechanisms in patients with Behcet's syndrome. J Am Coll Cardiol
37: 517-520
[Abstract][Full Text]
Hensel, M., Breitbart, A., Ho, A. D.
(2001). Autologous Hematopoietic Stem-Cell Transplantation for Behcet's Disease with Pulmonary Involvement. NEJM
344: 69-69
[Full Text]
Mandel, J., Mark, E. J.
(2000). Case 25-2000- A 69-Year-Old Woman with Pleuritic Pain and Pulmonary Arterial Obstruction. NEJM
343: 493-500
[Full Text]
Agca, M., Nzerue, C. M., Ehrlich, G. E., Drenth, J. P.H., Livneh, A., Haznedaroglu, I. C., Ozcebe, O. I., Dundar, S. V., Cheng, T. O., Sakane, T., Takeno, M., Suzuki, N.
(2000). Behcet's Disease. NEJM
342: 587-589
[Full Text]