Semin Respir Crit Care Med 2004; 25(5): 557-568
DOI: 10.1055/s-2004-836147
Copyright © 2004 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Behçet's Disease

Manae S. Kurokawa1 , 2 , Hideshi Yoshikawa1 , 2 , Noboru Suzuki1 , 2
  • 1Department of Immunology, St. Marianna University School of Medicine, Kanagawa, Japan
  • 2Departments of Immunology and Medicine, St. Marianna University School of Medicine, Kanagawa, Japan
Further Information

Publication History

Publication Date:
09 November 2004 (online)

Behçet's disease (BD) is a systemic disorder characterized by recurrent attacks of acute inflammation. Major symptoms are oral aphthous ulcers, uveitis, skin lesions, and genital ulcerations. Involvement of vessels, gastrointestinal (GI) tract, and central nervous system (CNS) is less frequent but is associated with a poor prognosis. Pulmonary complications of BD include aneurysms of the aorta, great vessels, or pulmonary arteries; arterial or venous thrombosis; pulmonary parenchymal changes; pleurisy, and intracardiac thrombosis. Hemoptysis caused by pulmonary artery aneurysms may lead to lethal hemorrhage. Recent advances in therapeutic strategies have improved the prognosis. In this review, the salient clinical and histopathological features of BD and treatment strategies are discussed.

REFERENCES

  • 1 Sakane T, Takeno M, Suzuki N, Inaba G. Current concepts: Behçet's disease.  N Engl J Med. 1999;  341 1284-1291
  • 2 Barnes C G, Yazici H. Behçet's syndrome.  Rheumatology (Oxford). 1999;  38 1171-1174
  • 3 Zouboulis C C, Kotter I, Djawari D et al.. Epidemiological features of Adamantiades-Behçet's disease in Germany and in Europe.  Yonsei Med J. 1997;  38 411-422
  • 4 Hamzaoui K, Ayed K, Slim A, Hamza M, Touraine J. Natural killer cell activity, interferon-gamma and antibodies to herpes viruses in patients with Behçet's disease.  Clin Exp Immunol. 1990;  79 28-34
  • 5 Lee S, Bang D, Cho Y H, Lee E S, Sohn S. Polymerase chain reaction reveals herpes simplex virus DNA in saliva of patients with Behçet's disease.  Arch Dermatol Res. 1996;  288 179-183
  • 6 Aoki K, Ohno S. Studies on the constitution and past history of patients with Behçet's disease.  Acta Soc Ophthalmol Jpn. 1972;  76 1608-1612
  • 7 Kaneko F, Takahashi Y, Muramatsu Y, Miura Y. Immunological studies on aphthous ulcer and erythema nodosum-like eruptions in Behçet's disease.  Br J Dermatol. 1985;  113 303-312
  • 8 Direskeneli H, Hasan A, Shinnick T et al.. Recognition of B-cell epitopes of the 65 kDa HSP in Behçet's disease.  Scand J Immunol. 1996;  43 464-471
  • 9 Tasci B, Direskeneli H, Serdaroglu P, Akman-Demir G, Eraksoy M, Saruhan-Direskeneli G. Humoral immune response to mycobacterial heat shock protein (hsp) 65 in the cerebrospinal fluid of neuro-Behçet patients.  Clin Exp Immunol. 1998;  113 100-104
  • 10 Kaneko S, Suzuki N, Yamashita N et al.. Characterization of T cells specific for an epitope of human 60-kD heat shock protein (hsp) in patients with Behçet's disease (BD) in Japan.  Clin Exp Immunol. 1997;  108 204-212
  • 11 Ergun T, Ince U, Eksioglu-Demiralp E et al.. HSP 60 expression in mucocutaneous lesions of Behçet's disease.  J Am Acad Dermatol. 2001;  45 904-909
  • 12 Kurokawa M S, Suzuki N. Behçet's disease.  Clin Exp Med. In press.
  • 13 Takeno M, Kariyone A, Yamashita N et al.. Excessive function of peripheral blood neutrophils from patients with Behçet's disease and from HLA-B51 transgenic mice.  Arthritis Rheum. 1995;  38 426-433
  • 14 Hamzaoui K, Hamzaoui A, Guemira F, Bessioud M, Hamza M, Ayed K. Cytokine profile in Behçet's disease patients: relationship with disease activity.  Scand J Rheumatol. 2002;  31 205-210
  • 15 Frassanito M A, Dammacco R, Cafforio P, Dammacco F. Th1 polarization of the immune response in Behçet's disease: a putative pathogenetic role of interleukin-12.  Arthritis Rheum. 1999;  42 1967-1974
  • 16 International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease.  Lancet. 1990;  335 1078-1080
  • 17 Matsumoto T, Uekusa T, Fukuda Y. Vasculo-Behçet's disease: a pathologic study of eight cases.  Hum Pathol. 1991;  22 45-51
  • 18 Sullivan E J, Hoffman G S. Pulmonary vasculitis.  Clin Chest Med. 1998;  19 759-776
  • 19 Erkan F, Gul A, Tasali E. Pulmonary manifestations of Behçet's disease.  Thorax. 2001;  56 572-578
  • 20 Gunen H, Evereklioglu C, Kosar F, Er H, Kizkin O. Thoracic involvement in Behçet's disease and its correlation with multiple parameters.  Lung. 2000;  178 161-170
  • 21 Saba D, Saricaoglu H, Bayram A S et al.. Arterial lesions in Behçet's disease.  Vasa. 2003;  32 75-81
  • 22 Akdag Kose A, Kayabali M, Sarica R, Saglik E, Azizlerli G. Pulmonary artery involvement in Behçet's disease.  Adv Exp Med Biol. 2003;  528 419-422
  • 23 Aksu K, Kocanaogullari H, Keser G et al.. A case of Behçet's disease with pulmonary arterial aneurysm and secondary amyloidosis.  Rheumatology (Oxford). 2002;  41 831-832
  • 24 Dikensoy O, Bayram N G, Filiz A. Massive haemoptysis in a young woman.  Postgrad Med J. 2002;  78 183 187-188
  • 25 Aktogu S, Erer O F, Urpek G, Soy O, Tibet G. Multiple pulmonary arterial aneurysms in Behçet's disease: clinical and radiologic remission after cyclophosphamide and corticosteroid therapy.  Respiration. 2002;  69 178-181
  • 26 Mahendran C, Singh P, Mani N B, Jindal S K. Successful treatment of pulmonary artery aneurysms secondary to possible Behçet's disease.  Respiration. 2002;  69 355-358
  • 27 Bozkurt A K. Embolisation in Behçet's disease.  Thorax. 2002;  57 469-470
  • 28 Ozcan H, Aytac S K, Yagmurlu B, Kaya A. Color Doppler examination of a regressing pulmonary artery pseudoaneurysm due to Behçet disease.  J Ultrasound Med. 2002;  21 697-700
  • 29 Cantasdemir M, Kantarci F, Mihmanli I et al.. Emergency endovascular management of pulmonary artery aneurysms in Behçet's disease: report of two cases and a review of the literature.  Cardiovasc Intervent Radiol. 2002;  25 533-537
  • 30 Acican T, Gurkan O U. Azathioprine-steroid combination therapy for pulmonary arterial aneurysms in Behçet's disease.  Rheumatol Int. 2001;  20 171-174
  • 31 Filiz A, Dikensoy O. Lethal aneurysm formation of pulmonary arteries in a woman with Behçet's disease.  Rheumatology(Oxford). 2000;  39 222-224
  • 32 Sekine K, Nakajima Y, Sawamoto S et al.. Vasculo-Behçet's disease with fatal massive hemoptysis.  Nihon Kokyuki Gakkai Zasshi. 1999;  37 135-139
  • 33 Berkan O, Ozturkcan S, Dogan K, Onen A, Basel H, Hatipogu A. Pulmonary arterial aneurysm in Behçet's disease.  J Eur Acad Dermatol Venereol. 1999;  13 140-141
  • 34 Celenk C, Celenk P, Akan H, Basoglu A. Pulmonary artery aneurysms due to Behçet's disease: MR imaging and digital subtraction angiography findings.  AJR Am J Roentgenol. 1999;  172 844-845
  • 35 Sasaki S h, Yasuda K, Takigami K, Shiiya N, Matsui Y, Sakuma M. Surgical experiences with peripheral arterial aneurysms due to vasculo-Behçet's disease.  J Cardiovasc Surg (Torino). 1998;  39 147-150
  • 36 Berkmen T. MR angiography of aneurysms in Behçet disease: a report of four cases.  J Comput Assist Tomogr. 1998;  22 202-206
  • 37 Gebitekin C, Yilmaz M, Senkaya I, Saba D, Sagdic K, Ozer G. Fatal haemoptysis due to pulmonary artery aneurysm in Behçet's disease.  Eur J Vasc Endovasc Surg. 1997;  13 233-236
  • 38 Gotor M A, Medrano J, Ruiz C, Alfonso E R, Guerrero J, de Gregorio M A. Pulmonary artery aneurysm and thrombosis caused by vasculitis in Behçet's disease.  Arch Bronconeumol (Spain). 1997;  33 591-593
  • 39 Wiatr E, Chmielewska B, Jotowicz E, Pawlicka L, Remiszewski P, Rowinska-Zakrzewska E. Behçet's disease with pulmonary vessel involvement.  Pneumonol Alergol Pol. 1997;  65 830-836
  • 40 Yassine N, el Meziane A, Alaoui-Yazidi A, Bartal M. Aneurysms of the pulmonary artery in Behçet disease: apropos of 5 new cases.  Rev Pneumol Clin. 1997;  53 42-48
  • 41 Lacombe P, Qanadli S D, Jondeau G et al.. Treatment of hemoptysis in Behçet syndrome with pulmonary and bronchial embolization.  J Vasc Interv Radiol. 1997;  8 1043-1047
  • 42 Cemri M, Erkan A, Ozdemir M, Cengel A. Behçet's disease with a large and free right atrial thrombus.  Eur J Echocardiogr. 2002;  3 233-235
  • 43 Dincer I, Dandachi R, Atmaca Y, Erol C, Caglar N, Oral D. A recurrent right heart thrombus in a patient with Behçet's disease.  Echocardiography. 2001;  18 15-18
  • 44 Hensel M, Breitbart A, Ho A D. Autologous hematopoietic stem-cell transplantation for Behçet's disease with pulmonary involvement.  N Engl J Med. 2001;  344 69
  • 45 Duchene F, Berthier S, de Wazieres B et al.. Behçet disease with cardiac and pulmonary manifestations.  Presse Med. 1998;  27 1674-1676
  • 46 Ceyran H, Akcali Y, Kahraman C. Surgical treatment of vasculo-Behçet's disease: a review of patients with concomitant multiple aneurysms and venous lesions.  Vasa. 2003;  32 149-153
  • 47 Duzgun N, Anil C, Ozer F, Acican T. The disappearance of pulmonary artery aneurysms and intracardiac thrombus with immunosuppressive treatment in a patient with Behçet's disease.  Clin Exp Rheumatol. 2002;  20 S56-S57
  • 48 Hassikou H, Bono W, Bahiri R, Abir S, Benomar M, Hassouni N H. Vascular involvement in Behçet's disease: two case reports.  Joint Bone Spine. 2002;  69 416-418
  • 49 Cohle S D, Colby T. Fatal hemoptysis from Behçet's disease in a child.  Cardiovasc Pathol. 2002;  11 296-299
  • 50 Ozatli D, Kav T, Haznedaroglu I C et al.. Cardiac and great vessel thrombosis in Behçet's disease.  Intern Med. 2001;  40 68-72
  • 51 Caterino U, Paciocco G, D'Auria D, Mazzarella G. Subpleural lung involvement in Behçet's disease: first localization of a systemic entity.  Monaldi Arch Chest Dis. 2000;  55 289-292
  • 52 Oz N, Sarper A, Erdogan A, Demircan A, Isin E. Video-assisted thoracic surgery for the management of pleural and pericardial effusion in Behçet's syndrome.  Tex Heart Inst J. 2000;  27 304-306
  • 53 Misgav M, Goldberg Y, Zeltser D, Eldor A, Berliner A S. Fatal pulmonary artery thrombosis in a patient with Behçet's disease, activated protein C resistance and hyperhomocystinemia.  Blood Coagul Fibrinolysis. 2000;  11 421-423
  • 54 Bozkurt M F, Demirkazik F B, Emri S, Caner B. Ventilation-perfusion lung scan and helical computed tomographic findings in a patient with Behçet's disease.  Clin Nucl Med. 2001;  26 721-722
  • 55 Yasuo M, Nagano S, Yazaki Y et al.. Pulmonary embolism due to right ventricular thrombus in a case of Behçet's disease.  Jpn Circ J. 1999;  63 909-911
  • 56 Gul A, Yilmazbayhan D, Buyukbabani N et al.. Organizing pneumonia associated with pulmonary artery aneurysms in Behçet's disease.  Rheumatology (Oxford). 1999;  38 1285-1289
  • 57 Vansteenkiste J, Van Haecke P, Demedts M. Long-term treatment with cyclosporin A and coumarin in pulmonary thromboembolic Behçet's disease.  Monaldi Arch Chest Dis. 1998;  53 142-143
  • 58 Basak M, Gul S, Yazgan Y et al.. A case of rapidly progressive pulmonary aneurysm as a rare complication of Behçet's syndrome-a case report.  Angiology. 1998;  49 403-408
  • 59 Greene R M, Saleh A, Taylor A K et al.. Noninvasive assessment of bleeding pulmonary artery aneurysms due to Behçet disease.  Eur Radiol. 1998;  8 359-363
  • 60 Huong D L, Dolmazon C, De Zuttere D, Wechsler B, Godeau P, Piette J C. Complete recovery of right intraventricular thrombus and pulmonary arteritis in Behçet's disease.  Br J Rheumatol. 1997;  36 130-132
  • 61 Yoshimura H, Ishii J, Watanabe N et al.. A case of cardiovascular Behçet's disease detected as multiple nodular shadows on chest x-ray.  Nihon Kyobu Shikkan Gakkai Zasshi. 1997;  35 1074-1079
  • 62 Hirose H, Takagi M, Noguchi M et al.. Coronary revascularization and abdominal aortic aneurysm repair in a patient with Behçet's diseases.  J Cardiovasc Surg (Torino). 1998;  39 751-755
  • 63 Mori K, Yoshinouchi T, Ohtsuki Y. Behçet's disease with acute inflammatory foci and localized secondary organizing pneumonia.  Nihon Kokyuki Gakkai Zasshi. 1998;  36 513-518
  • 64 Nonaka K, Makuuchi H, Naruse Y, Kobayashi T, Goto M, Yamamoto T. Pseudoaneurysm of aortic arch and rupture into pericardium, a case report of successful surgical management.  Jpn J Thorac Cardiovasc Surg. 1998;  46 772-776
  • 65 Okita Y, Ando M, Minatoya K, Kitamura S, Matsuo H. Multiple pseudoaneurysms of the aortic arch, right subclavian artery, and abdominal aorta in a patient with Behçet's disease.  J Vasc Surg. 1998;  28 723-726
  • 66 Han D S, Kim J B, Lee O Y, Sohn J H, Park K N, Park C K. A case of Behçet's syndrome with supeior vena cava syndrome.  Korean J Intern Med. 1998;  13 72-75
  • 67 Mogulkoc N, Burgess M I, Bishop P W. Intracardiac thrombus in Behçet's disease: a systematic review.  Chest. 2000;  118 479-487
  • 68 Erkan F, Kiyan E, Tunaci A. Pulmonary complications of Behçet's disease.  Clin Chest Med. 2002;  23 493-503
  • 69 Tunaci M, Ozkorkmaz B, Tunaci A, Gul A, Engin G, Acunas B. CT findings of pulmonary artery aneurysms during treatment for Behçet's disease.  AJR Am J Roentgenol. 1999;  172 729-733
  • 70 Caglar M, Ergun E, Emri S. 99Tcm-MAA lung scintigraphy in patients with Behçet's disease: its value and correlation with clinical course and other diagnostic modalities.  Nucl Med Commun. 2000;  21 171-179
  • 71 Unlu M, Akincioglu C, Yamac K, Onder M. Pulmonary involvement in Behçet's disease: evaluation of 123I-MIBG retention.  Nucl Med Commun. 2001;  22 1083-1088
  • 72 Hamzaoui A, Hamzaoui K, Chabbou A, Ayed K. Endothelin-1 expression in serum and bronchoalveolar lavage from patients with active Behçet's disease.  Br J Rheumatol. 1996;  35 357-358
  • 73 Leiba M, Sidi Y, Gur H, Leiba A, Ehrenfeld M. Behçet's disease and thrombophilia.  Ann Rheum Dis. 2001;  60 1081-1085
  • 74 Lee Y J, Kang S W, Yang J I et al.. Coagulation parameters and plasma total homocysteine levels in Behçet's disease.  Thromb Res. 2002;  106 19-24
  • 75 Espinosa G, Font J, Tassies D et al.. Vascular involvement in Behçet's disease: relation with thrombophilic factors, coagulation activation, and thrombomodulin.  Am J Med. 2002;  112 37-43
  • 76 Fusegawa H, Ichikawa Y, Tanaka Y et al.. Blood coagulation and fibrinolysis in patients with Behçet's disease.  Rinsho Byori. 1991;  39 509-516
  • 77 Sengul N, Demirer S, Yerdel M A et al.. Comparison of coagulation parameters for healthy subjects and Behçet disease patients with and without vascular involvement.  World J Surg. 2000;  24 1584-1588
  • 78 Famularo G, Antonelli S, Barracchini A, Menichelli M, Nicotra G C, Minisola G. Catastrophic antiphospholipid syndrome in a patient with Behçet's disease.  Scand J Rheumatol. 2002;  31 100-102
  • 79 Thomas-Golbanov C, Sridharan S. Novel therapies in vasculitis.  Expert Opin Investig Drugs. 2001;  10 1279-1289
  • 80 Langford C A, Talar-Williams C, Barron K S, Sneller M C. Use of a cyclophosphamide-induction methotrexate-maintenance regimen for the treatment of Wegener's granulomatosis: extended follow-up and rate of relapse.  Am J Med. 2003;  114 463-469

Noboru SuzukiM.D. Ph.D. 

Departments of Immunology and Medicine, St. Marianna University School of Medicine

2-16-1, Sugao, Miyamae-ku, Kawasaki

Kanagawa 216-8511, Japan

Email: n3suzuki@marianna-u.ac.jp