Skip to main content
Postgraduate Medical Journal logoLink to Postgraduate Medical Journal
. 2000 Oct;76(900):629–637. doi: 10.1136/pmj.76.900.629

Behçet's disease

V Kontogiannis 1, R Powell 1
PMCID: PMC1741755  PMID: 11009577

Abstract

Behçet's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital ulceration, uveitis, skin manifestations, arthritis, neurological involvement, and a tendency to thrombosis. It has a worldwide distribution but is prevalent in Japan, the Middle East, and some Mediterranean countries. International diagnostic criteria have been proposed, however diagnosis can be problematical, particularly if the typical ulcers are not obvious at presentation. Treatment is challenging, must be tailored to the pattern of organ involvement for each patient and often requires combination therapies.


Keywords: Behçet's disease; oral ulcers; uveitis; immunosuppressants

Full Text

The Full Text of this article is available as a PDF (232.4 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aitchison R., Chu P., Cater D. R., Harris R. J., Powell R. J. Defective fibrinolysis in Behçet's syndrome: significance and possible mechanisms. Ann Rheum Dis. 1989 Jul;48(7):590–593. doi: 10.1136/ard.48.7.590. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Akoğlu T. F., Direskeneli H., Yazici H., Lawrence R. TNF, soluble IL-2R and soluble CD-8 in Behçet's disease. J Rheumatol. 1990 Aug;17(8):1107–1108. [PubMed] [Google Scholar]
  3. Akpolat I., Akpolat T., Danaci M., Bariş Y. S., Kaya N., Kandemir B. Behçet's disease and amyloidosis. Review of the literature. Scand J Rheumatol. 1997;26(6):477–479. doi: 10.3109/03009749709065723. [DOI] [PubMed] [Google Scholar]
  4. Aktulga E., Altaç M., Müftüoglu A., Ozyazgan Y., Pazarli H., Tüzün Y., Yalçin B., Yazici H., Yurdakul S. A double blind study of colchicine in Behçet's disease. Haematologica. 1980 Jun;65(3):399–402. [PubMed] [Google Scholar]
  5. Akutsu Y., Itami N., Tanaka M., Kusunoki Y., Tochimaru H., Takekoshi Y. IgA nephritis in Behçet's disease: case report and review of the literature. Clin Nephrol. 1990 Aug;34(2):52–55. [PubMed] [Google Scholar]
  6. Arber N., Klein T., Meiner Z., Pras E., Weinberger A. Close association of HLA-B51 and B52 in Israeli patients with Behçet's syndrome. Ann Rheum Dis. 1991 Jun;50(6):351–353. doi: 10.1136/ard.50.6.351. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Atmaca L. S., Idil A., Batioĝlu F. A descriptive study on Behçet's disease. Acta Ophthalmol Scand. 1996 Aug;74(4):403–406. doi: 10.1111/j.1600-0420.1996.tb00718.x. [DOI] [PubMed] [Google Scholar]
  8. Bayraktar Y., Balkanci F., Bayraktar M., Calguneri M. Budd-Chiari syndrome: a common complication of Behçet's disease. Am J Gastroenterol. 1997 May;92(5):858–862. [PubMed] [Google Scholar]
  9. Binder A. I., Graham E. M., Sanders M. D., Dinning W., James D. G., Denman A. M. Cyclosporin A in the treatment of severe Behçet's uveitis. Br J Rheumatol. 1987 Aug;26(4):285–291. doi: 10.1093/rheumatology/26.4.285. [DOI] [PubMed] [Google Scholar]
  10. Bird Stewart J. A. Genetic analysis of families of patients with Behçet's syndrome: data incompatible with autosomal recessive inheritance. Ann Rheum Dis. 1986 Apr;45(4):265–268. doi: 10.1136/ard.45.4.265. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Budak-Alpdogan T., Demirçay, Alpdogan O., Direskeneli H., Ergun T., Oztürk A., Günay A., Yavuz S., Uskent N., Akoğlu T. Skin hyperreactivity of Behçet's patients (pathergy reaction) is also positive in interferon alpha-treated chronic myeloid leukaemia patients, indicating similarly altered neutrophil functions in both disorders. Br J Rheumatol. 1998 Nov;37(11):1148–1151. doi: 10.1093/rheumatology/37.11.1148. [DOI] [PubMed] [Google Scholar]
  12. Calgüneri M., Ertenli I., Kiraz S., Erman M., Celik I. Effect of prophylactic benzathine penicillin on mucocutaneous symptoms of Behçet's disease. Dermatology. 1996;192(2):125–128. doi: 10.1159/000246336. [DOI] [PubMed] [Google Scholar]
  13. Calgüneri M., Kiraz S., Ertenli I., Benekli M., Karaarslan Y., Celik I. The effect of prophylactic penicillin treatment on the course of arthritis episodes in patients with Behçet's disease. A randomized clinical trial. Arthritis Rheum. 1996 Dec;39(12):2062–2065. doi: 10.1002/art.1780391216. [DOI] [PubMed] [Google Scholar]
  14. Chamberlain M. A. Behcet's syndrome in 32 patients in Yorkshire. Ann Rheum Dis. 1977 Dec;36(6):491–499. doi: 10.1136/ard.36.6.491. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Chikamori T., Doi Y. L., Yonezawa Y., Takata J., Kawamura M., Ozawa T. Aortic regurgitation secondary to Behçet's disease. A case report and review of the literature. Eur Heart J. 1990 Jun;11(6):572–576. doi: 10.1093/oxfordjournals.eurheartj.a059752. [DOI] [PubMed] [Google Scholar]
  16. Chun S. I., Su W. P., Lee S., Rogers R. S., 3rd Erythema nodosum-like lesions in Behçet's syndrome: a histopathologic study of 30 cases. J Cutan Pathol. 1989 Oct;16(5):259–265. doi: 10.1111/j.1600-0560.1989.tb00049.x. [DOI] [PubMed] [Google Scholar]
  17. Davies P. G., Fordham J. N., Kirwan J. R., Barnes C. G., Dinning W. J. The pathergy test and Behçet's syndrome in Britain. Ann Rheum Dis. 1984 Feb;43(1):70–73. doi: 10.1136/ard.43.1.70. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Du L. T., Fain O., Wechsler B., Cochereau I., Hoang P. L., Souillem J., Piette J. C., Blétry O., Godeau P. Intérêt des "bolus" de cyclophosphamide dans la maladie de Behçet. Expérience de 17 cas. Presse Med. 1990 Sep 15;19(29):1355–1358. [PubMed] [Google Scholar]
  19. Eglin R. P., Lehner T., Subak-Sharpe J. H. Detection of RNA complementary to herpes-simplex virus in mononuclear cells from patients with Behçet's syndrome and recurrent oral ulcers. Lancet. 1982 Dec 18;2(8312):1356–1361. doi: 10.1016/s0140-6736(82)91268-5. [DOI] [PubMed] [Google Scholar]
  20. Elidan J., Levi H., Cohen E., BenEzra D. Effect of cyclosporine A on the hearing loss in Behçet's disease. Ann Otol Rhinol Laryngol. 1991 Jun;100(6):464–468. doi: 10.1177/000348949110000607. [DOI] [PubMed] [Google Scholar]
  21. Ergun T., Gürbüz O., Dogusoy G., Mat C., Yazici H. Histopathologic features of the spontaneous pustular lesions of Behçet's syndrome. Int J Dermatol. 1998 Mar;37(3):194–196. doi: 10.1046/j.1365-4362.1998.00276.x. [DOI] [PubMed] [Google Scholar]
  22. FEIGENBAUM A. Description of Behçet's syndrome in the Hippocratic third book of endemic diseases. Br J Ophthalmol. 1956 Jun;40(6):355–357. doi: 10.1136/bjo.40.6.355. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Feder H. M., Jr, Bialecki C. A. Periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Pediatr Infect Dis J. 1989 Mar;8(3):186–187. [PubMed] [Google Scholar]
  24. Fresko I., Yazici H., Bayramiçli M., Yurdakul S., Mat C. Effect of surgical cleaning of the skin on the pathergy phenomenon in Behçet's syndrome. Ann Rheum Dis. 1993 Aug;52(8):619–620. doi: 10.1136/ard.52.8.619. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Gül A., Esin S., Dilsen N., Koniçe M., Wigzell H., Biberfeld P. Immunohistology of skin pathergy reaction in Behçet's disease. Br J Dermatol. 1995 Jun;132(6):901–907. doi: 10.1111/j.1365-2133.1995.tb16946.x. [DOI] [PubMed] [Google Scholar]
  26. Hamuryudan V., Mat C., Saip S., Ozyazgan Y., Siva A., Yurdakul S., Zwingenberger K., Yazici H. Thalidomide in the treatment of the mucocutaneous lesions of the Behçet syndrome. A randomized, double-blind, placebo-controlled trial. Ann Intern Med. 1998 Mar 15;128(6):443–450. doi: 10.7326/0003-4819-128-6-199803150-00004. [DOI] [PubMed] [Google Scholar]
  27. Hamuryudan V., Ozyazgan Y., Hizli N., Mat C., Yurdakul S., Tüzün Y., Senocak M., Yazici H. Azathioprine in Behcet's syndrome: effects on long-term prognosis. Arthritis Rheum. 1997 Apr;40(4):769–774. doi: 10.1002/art.1780400425. [DOI] [PubMed] [Google Scholar]
  28. Hamuryudan V., Yurdakul S., Moral F., Numan F., Tüzün H., Tüzüner N., Mat C., Tüzün Y., Ozyazgan Y., Yazïci H. Pulmonary arterial aneurysms in Behçet's syndrome: a report of 24 cases. Br J Rheumatol. 1994 Jan;33(1):48–51. doi: 10.1093/rheumatology/33.1.48. [DOI] [PubMed] [Google Scholar]
  29. Hamzaoui K., Ayed K., Hamza M., Touraine J. L. Natural killer cells in Behçet's disease. Clin Exp Immunol. 1988 Jan;71(1):126–131. [PMC free article] [PubMed] [Google Scholar]
  30. Hasan A., Fortune F., Wilson A., Warr K., Shinnick T., Mizushima Y., van der Zee R., Stanford M. R., Sanderson J., Lehner T. Role of gamma delta T cells in pathogenesis and diagnosis of Behcet's disease. Lancet. 1996 Mar 23;347(9004):789–794. doi: 10.1016/s0140-6736(96)90868-5. [DOI] [PubMed] [Google Scholar]
  31. Hazleman B. L. Rheumatic disorders of the eye and the various structures involved. Br J Rheumatol. 1996 Mar;35(3):258–268. doi: 10.1093/rheumatology/35.3.258. [DOI] [PubMed] [Google Scholar]
  32. Hemmen T., Perez-Canto A., Distler A., Offermann G., Braun J. IgA nephropathy in a patient with Behçet's syndrome--case report and review of literature. Br J Rheumatol. 1997 Jun;36(6):696–699. doi: 10.1093/rheumatology/36.6.696. [DOI] [PubMed] [Google Scholar]
  33. Hirohata S., Oka H., Mizushima Y. Streptococcal-related antigens stimulate production of IL6 and interferon-gamma by T cells from patients with Behcet's disease. Cell Immunol. 1992 Apr;140(2):410–419. doi: 10.1016/0008-8749(92)90207-6. [DOI] [PubMed] [Google Scholar]
  34. Hirohata S., Suda H., Hashimoto T. Low-dose weekly methotrexate for progressive neuropsychiatric manifestations in Behcet's disease. J Neurol Sci. 1998 Aug 14;159(2):181–185. doi: 10.1016/s0022-510x(98)00165-8. [DOI] [PubMed] [Google Scholar]
  35. Huong D. L., Wechsler B., Papo T., de Zuttere D., Bletry O., Hernigou A., Delcourt A., Godeau P., Piette J. C. Endomyocardial fibrosis in Behçet's disease. Ann Rheum Dis. 1997 Mar;56(3):205–208. doi: 10.1136/ard.56.3.205. [DOI] [PMC free article] [PubMed] [Google Scholar]
  36. Imai H., Motegi M., Mizuki N., Ohtani H., Komatsuda A., Hamai K., Miura A. B. Mouth and genital ulcers with inflamed cartilage (MAGIC syndrome): a case report and literature review. Am J Med Sci. 1997 Nov;314(5):330–332. doi: 10.1097/00000441-199711000-00010. [DOI] [PubMed] [Google Scholar]
  37. Ioakimidis D., Georganas C., Panagoulis C., Gournizakis A., Iliopoulos A., Kremastinos D., Kontomerkos T. A case of Adamantiadis-Behçet's syndrome presenting as myocardial infarction. Clin Exp Rheumatol. 1993 Mar-Apr;11(2):183–186. [PubMed] [Google Scholar]
  38. Jankowski J., Crombie I., Jankowski R. Behçet's syndrome in Scotland. Postgrad Med J. 1992 Jul;68(801):566–570. doi: 10.1136/pgmj.68.801.566. [DOI] [PMC free article] [PubMed] [Google Scholar]
  39. Jorizzo J. L., White W. L., Wise C. M., Zanolli M. D., Sherertz E. F. Low-dose weekly methotrexate for unusual neutrophilic vascular reactions: cutaneous polyarteritis nodosa and Behçet's disease. J Am Acad Dermatol. 1991 Jun;24(6 Pt 1):973–978. doi: 10.1016/0190-9622(91)70156-v. [DOI] [PubMed] [Google Scholar]
  40. Kaklamani V. G., Vaiopoulos G., Kaklamanis P. G. Behçet's Disease. Semin Arthritis Rheum. 1998 Feb;27(4):197–217. doi: 10.1016/s0049-0172(98)80001-2. [DOI] [PubMed] [Google Scholar]
  41. Kaneko F., Oyama N., Nishibu A. Streptococcal infection in the pathogenesis of Behçet's disease and clinical effects of minocycline on the disease symptoms. Yonsei Med J. 1997 Dec;38(6):444–454. doi: 10.3349/ymj.1997.38.6.444. [DOI] [PubMed] [Google Scholar]
  42. Kansu E., Sahin G., Sahin F., Sivri B., Sayek I., Batman F. Impaired prostacyclin synthesis by vessel walls in Behçet's disease. Lancet. 1986 Nov 15;2(8516):1154–1154. doi: 10.1016/s0140-6736(86)90552-0. [DOI] [PubMed] [Google Scholar]
  43. Kim H. A., Choi K. W., Song Y. W. Arthropathy in Behçet's disease. Scand J Rheumatol. 1997;26(2):125–129. doi: 10.3109/03009749709115831. [DOI] [PubMed] [Google Scholar]
  44. Koné-Paut I., Yurdakul S., Bahabri S. A., Shafae N., Ozen S., Ozdogan H., Bernard J. L. Clinical features of Behçet's disease in children: an international collaborative study of 86 cases. J Pediatr. 1998 Apr;132(4):721–725. doi: 10.1016/s0022-3476(98)70368-3. [DOI] [PubMed] [Google Scholar]
  45. Koç Y., Güllü I., Akpek G., Akpolat T., Kansu E., Kiraz S., Batman F., Kansu T., Balkanci F., Akkaya S. Vascular involvement in Behçet's disease. J Rheumatol. 1992 Mar;19(3):402–410. [PubMed] [Google Scholar]
  46. Krause I., Uziel Y., Guedj D., Mukamel M., Harel L., Molad Y., Weinberger A. Childhood Behçet's disease: clinical features and comparison with adult-onset disease. Rheumatology (Oxford) 1999 May;38(5):457–462. doi: 10.1093/rheumatology/38.5.457. [DOI] [PubMed] [Google Scholar]
  47. Lee E. S., Bang D., Lee S. Dermatologic manifestation of Behçet's disease. Yonsei Med J. 1997 Dec;38(6):380–389. doi: 10.3349/ymj.1997.38.6.380. [DOI] [PubMed] [Google Scholar]
  48. Lee M. S., Barnetson R. S. Sweet's syndrome associated with Behçet's disease. Australas J Dermatol. 1996 May;37(2):99–101. doi: 10.1111/j.1440-0960.1996.tb01015.x. [DOI] [PubMed] [Google Scholar]
  49. Lehner T., Lavery E., Smith R., van der Zee R., Mizushima Y., Shinnick T. Association between the 65-kilodalton heat shock protein, Streptococcus sanguis, and the corresponding antibodies in Behçet's syndrome. Infect Immun. 1991 Apr;59(4):1434–1441. doi: 10.1128/iai.59.4.1434-1441.1991. [DOI] [PMC free article] [PubMed] [Google Scholar]
  50. Levinsky R. J., Lehner T. Circulating soluble immume complexes in recurrent oral ulceration and Behçet's syndrome. Clin Exp Immunol. 1978 May;32(2):193–198. [PMC free article] [PubMed] [Google Scholar]
  51. Lilford R. J., Tindall V. R., Batchelor A. G. Post-surgical pyoderma gangrenosum of the vaginal vault associated with ulcerative colitis and Behçet's disease; a case report. Eur J Obstet Gynecol Reprod Biol. 1989 Apr;31(1):93–94. doi: 10.1016/0028-2243(89)90030-0. [DOI] [PubMed] [Google Scholar]
  52. Main D. M., Chamberlain M. A. Clinical differentiation of oral ulceration in Behçet's disease. Br J Rheumatol. 1992 Nov;31(11):767–770. doi: 10.1093/rheumatology/31.11.767. [DOI] [PubMed] [Google Scholar]
  53. Manzi S., Meilahn E. N., Rairie J. E., Conte C. G., Medsger T. A., Jr, Jansen-McWilliams L., D'Agostino R. B., Kuller L. H. Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham Study. Am J Epidemiol. 1997 Mar 1;145(5):408–415. doi: 10.1093/oxfordjournals.aje.a009122. [DOI] [PubMed] [Google Scholar]
  54. Marsal S., Falgá C., Simeon C. P., Vilardell M., Bosch J. A. Behçet's disease and pregnancy relationship study. Br J Rheumatol. 1997 Feb;36(2):234–238. doi: 10.1093/rheumatology/36.2.234. [DOI] [PubMed] [Google Scholar]
  55. Masuda K., Nakajima A., Urayama A., Nakae K., Kogure M., Inaba G. Double-masked trial of cyclosporin versus colchicine and long-term open study of cyclosporin in Behçet's disease. Lancet. 1989 May 20;1(8647):1093–1096. doi: 10.1016/s0140-6736(89)92381-7. [DOI] [PubMed] [Google Scholar]
  56. Mishima S., Masuda K., Izawa Y., Mochizuki M., Namba K. The eighth Frederick H. Verhoeff Lecture. presented by saiichi mishima, MD Behçet's disease in Japan: ophthalmologic aspects. Trans Am Ophthalmol Soc. 1979;77:225–279. [PMC free article] [PubMed] [Google Scholar]
  57. Mizuki N., Inoko H., Ando H., Nakamura S., Kashiwase K., Akaza T., Fujino Y., Masuda K., Takiguchi M., Ohno S. Behçet's disease associated with one of the HLA-B51 subantigens, HLA-B* 5101. Am J Ophthalmol. 1993 Oct 15;116(4):406–409. doi: 10.1016/s0002-9394(14)71396-0. [DOI] [PubMed] [Google Scholar]
  58. Mizuki N., Inoko H., Ohno S. Molecular genetics (HLA) of Behçet's disease. Yonsei Med J. 1997 Dec;38(6):333–349. doi: 10.3349/ymj.1997.38.6.333. [DOI] [PubMed] [Google Scholar]
  59. Mizuki N., Ohno S., Ando H., Chen L., Palimeris G. D., Stavropoulos-Ghiokas E., Ishihara M., Goto K., Nakamura S., Shindo Y. A strong association between HLA-B*5101 and Behçet's disease in Greek patients. Tissue Antigens. 1997 Jul;50(1):57–60. doi: 10.1111/j.1399-0039.1997.tb02835.x. [DOI] [PubMed] [Google Scholar]
  60. Mizushima Y., Matsuda T., Hoshi K., Ohno S. Induction of Behçet's disease symptoms after dental treatment and streptococcal antigen skin test. J Rheumatol. 1988 Jun;15(6):1029–1030. [PubMed] [Google Scholar]
  61. Moorthy R. S., Inomata H., Rao N. A. Vogt-Koyanagi-Harada syndrome. Surv Ophthalmol. 1995 Jan-Feb;39(4):265–292. doi: 10.1016/s0039-6257(05)80105-5. [DOI] [PubMed] [Google Scholar]
  62. Morelli S., Perrone C., Ferrante L., Sgreccia A., Priori R., Voci P., Accorinti M., Pivetti-Pezzi P., Valesini G. Cardiac involvement in Behçet's disease. Cardiology. 1997 Nov-Dec;88(6):513–517. doi: 10.1159/000177401. [DOI] [PubMed] [Google Scholar]
  63. Nishiura K., Kotake S., Ichiishi A., Matsuda H. Familial occurrence of Behçet's disease. Jpn J Ophthalmol. 1996;40(2):255–259. [PubMed] [Google Scholar]
  64. O'Duffy J. D., Robertson D. M., Goldstein N. P. Chlorambucil in the treatment of uveitis and meningoencephalitis of Behçet's disease. Am J Med. 1984 Jan;76(1):75–84. doi: 10.1016/0002-9343(84)90753-8. [DOI] [PubMed] [Google Scholar]
  65. Ohno S., Ohguchi M., Hirose S., Matsuda H., Wakisaka A., Aizawa M. Close association of HLA-Bw51 with Behçet's disease. Arch Ophthalmol. 1982 Sep;100(9):1455–1458. doi: 10.1001/archopht.1982.01030040433013. [DOI] [PubMed] [Google Scholar]
  66. Ozyazgan Y., Yurdakul S., Yazici H., Tüzün B., Işimen A., Tüzün Y., Aktunç T., Pazarli H., Hamuryudan V., Müftüoğlu A. Low dose cyclosporin A versus pulsed cyclophosphamide in Behçet's syndrome: a single masked trial. Br J Ophthalmol. 1992 Apr;76(4):241–243. doi: 10.1136/bjo.76.4.241. [DOI] [PMC free article] [PubMed] [Google Scholar]
  67. Pervin K., Childerstone A., Shinnick T., Mizushima Y., van der Zee R., Hasan A., Vaughan R., Lehner T. T cell epitope expression of mycobacterial and homologous human 65-kilodalton heat shock protein peptides in short term cell lines from patients with Behçet's disease. J Immunol. 1993 Aug 15;151(4):2273–2282. [PubMed] [Google Scholar]
  68. Powell R. J., Dunstan S. Immunopathology of Behçet's disease. Postgrad Med J. 1991 Jun;67(788):503–505. doi: 10.1136/pgmj.67.788.503. [DOI] [PMC free article] [PubMed] [Google Scholar]
  69. Powell R. J., Gardner-Medwin J. M. Guideline for the clinical use and dispensing of thalidomide. Postgrad Med J. 1994 Dec;70(830):901–904. doi: 10.1136/pgmj.70.830.901. [DOI] [PMC free article] [PubMed] [Google Scholar]
  70. Rogers R. S., 3rd Recurrent aphthous stomatitis: clinical characteristics and associated systemic disorders. Semin Cutan Med Surg. 1997 Dec;16(4):278–283. doi: 10.1016/s1085-5629(97)80017-x. [DOI] [PubMed] [Google Scholar]
  71. Rustin M. H., Gilkes J. J., Robinson T. W. Pyoderma gangrenosum associated with Behçet's disease: treatment with thalidomide. J Am Acad Dermatol. 1990 Nov;23(5 Pt 1):941–944. doi: 10.1016/s0190-9622(08)80705-0. [DOI] [PubMed] [Google Scholar]
  72. Sakane T., Mochizuki M., Inaba G., Masuda K. [A phase II study of FK506 (tacrolimus) on refractory uveitis associated with Behçet's disease and allied conditions]. Ryumachi. 1995 Oct;35(5):802–813. [PubMed] [Google Scholar]
  73. Schibler A., Birrer P., Vella S. PFAPA-Syndrom: periodisches Fieber, Adenitis, Pharyngitis und aphthöse Stomatitis. Schweiz Med Wochenschr. 1997 Aug 5;127(31-32):1280–1284. [PubMed] [Google Scholar]
  74. Sensi A., Gavioli R., Spisani S., Balboni A., Melchiorri L., Menicucci A., Palumbo G., Traniello S., Baricordi O. R. HLA B51 antigen associated with neutrophil hyper-reactivity. Dis Markers. 1991 Nov-Dec;9(6):327–331. [PubMed] [Google Scholar]
  75. Serdaroğlu P. Behçet's disease and the nervous system. J Neurol. 1998 Apr;245(4):197–205. doi: 10.1007/s004150050205. [DOI] [PubMed] [Google Scholar]
  76. Siepmann M., Kirch W. Coronary anomaly in Behçet's syndrome. Rheumatol Int. 1997;17(1):39–42. doi: 10.1007/pl00006848. [DOI] [PubMed] [Google Scholar]
  77. Sloper C. M., Powell R. J., Dua H. S. Tacrolimus (FK506) in the treatment of posterior uveitis refractory to cyclosporine. Ophthalmology. 1999 Apr;106(4):723–728. doi: 10.1016/S0161-6420(99)90156-2. [DOI] [PubMed] [Google Scholar]
  78. Stanford M. R., Kasp E., Whiston R., Hasan A., Todryk S., Shinnick T., Mizushima Y., Dumonde D. C., van der Zee R., Lehner T. Heat shock protein peptides reactive in patients with Behçet's disease are uveitogenic in Lewis rats. Clin Exp Immunol. 1994 Aug;97(2):226–231. doi: 10.1111/j.1365-2249.1994.tb06072.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  79. Studd M., McCance D. J., Lehner T. Detection of HSV-1 DNA in patients with Behçet's syndrome and in patients with recurrent oral ulcers by the polymerase chain reaction. J Med Microbiol. 1991 Jan;34(1):39–43. doi: 10.1099/00222615-34-1-39. [DOI] [PubMed] [Google Scholar]
  80. Takeno M., Kariyone A., Yamashita N., Takiguchi M., Mizushima Y., Kaneoka H., Sakane T. Excessive function of peripheral blood neutrophils from patients with Behçet's disease and from HLA-B51 transgenic mice. Arthritis Rheum. 1995 Mar;38(3):426–433. doi: 10.1002/art.1780380321. [DOI] [PubMed] [Google Scholar]
  81. Turan B., Gallati H., Erdi H., Gürler A., Michel B. A., Villiger P. M. Systemic levels of the T cell regulatory cytokines IL-10 and IL-12 in Bechçet's disease; soluble TNFR-75 as a biological marker of disease activity. J Rheumatol. 1997 Jan;24(1):128–132. [PubMed] [Google Scholar]
  82. Uslu T., Erem C., Tosun M., Deger O. Plasma endothelin-1 levels in Behçet's disease. Clin Rheumatol. 1997 Jan;16(1):59–61. doi: 10.1007/BF02238764. [DOI] [PubMed] [Google Scholar]
  83. Uysal H., Vahaboğlu H., Inan L., Vahaboğlu G. Acute febrile neutrophilic dermatosis (Sweet's syndrome) in neuro-Behçet's disease. Clin Neurol Neurosurg. 1993 Dec;95(4):319–322. doi: 10.1016/0303-8467(93)90109-t. [DOI] [PubMed] [Google Scholar]
  84. Whitcup S. M., Salvo E. C., Jr, Nussenblatt R. B. Combined cyclosporine and corticosteroid therapy for sight-threatening uveitis in Behçet's disease. Am J Ophthalmol. 1994 Jul 15;118(1):39–45. doi: 10.1016/s0002-9394(14)72840-5. [DOI] [PubMed] [Google Scholar]
  85. Wray D., Graykowski E. A., Notkins A. L. Role of mucosal injury in initiating recurrent aphthous stomatitis. Br Med J (Clin Res Ed) 1981 Dec 12;283(6306):1569–1570. doi: 10.1136/bmj.283.6306.1569. [DOI] [PMC free article] [PubMed] [Google Scholar]
  86. Yamashita N., Kaneoka H., Kaneko S., Takeno M., Oneda K., Koizumi H., Kogure M., Inaba G., Sakane T. Role of gammadelta T lymphocytes in the development of Behçet's disease. Clin Exp Immunol. 1997 Feb;107(2):241–247. doi: 10.1111/j.1365-2249.1997.274-ce1159.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  87. Yavuz S., Fresko I., Hamuryudan V., Yurdakul S., Yazici H. Fibromyalgia in Behçet's syndrome. J Rheumatol. 1998 Nov;25(11):2219–2220. [PubMed] [Google Scholar]
  88. Yazici H., Başaran G., Hamuryudan V., Hizli N., Yurdakul S., Mat C., Tüzün Y., Ozyazgan Y., Dimitriyadis I. The ten-year mortality in Behçet's syndrome. Br J Rheumatol. 1996 Feb;35(2):139–141. doi: 10.1093/rheumatology/35.2.139. [DOI] [PubMed] [Google Scholar]
  89. Yazici H., Chamberlain M. A., Tüzün Y., Yurdakul S., Müftüoglu A. A comparative study of the pathergy reaction among Turkish and British patients with Behçet's disease. Ann Rheum Dis. 1984 Feb;43(1):74–75. doi: 10.1136/ard.43.1.74. [DOI] [PMC free article] [PubMed] [Google Scholar]
  90. Yazici H., Tüzün Y., Pazarli H., Yurdakul S., Ozyazgan Y., Ozdoğan H., Serdaroğlu S., Ersanli M., Ulkü B. Y., Müftüoğlu A. U. Influence of age of onset and patient's sex on the prevalence and severity of manifestations of Behçet's syndrome. Ann Rheum Dis. 1984 Dec;43(6):783–789. doi: 10.1136/ard.43.6.783. [DOI] [PMC free article] [PubMed] [Google Scholar]
  91. Yazici H., Tüzün Y., Pazarli H., Yurdakul S., Yalçin B., Müftüoglu A. Behçet's disease as seen in Turkey. Haematologica. 1980 Jun;65(3):381–383. [PubMed] [Google Scholar]
  92. Yazici H., Yurdakul S., Hamuryudan V. Behçet's syndrome. Curr Opin Rheumatol. 1999 Jan;11(1):53–57. doi: 10.1097/00002281-199901000-00009. [DOI] [PubMed] [Google Scholar]
  93. Yoshikawa K., Kotake S., Matsuda H. [Behçet's disease and streptococcal antigens]. Nippon Ganka Gakkai Zasshi. 1996 Mar;100(3):173–180. [PubMed] [Google Scholar]
  94. Yurdakul S., Yazici H., Tüzün Y., Pazarli H., Yalçin B., Altaç M., Ozyazgan Y., Tüzüner N., Müftüoğlu A. The arthritis of Behçet's disease: a prospective study. Ann Rheum Dis. 1983 Oct;42(5):505–515. doi: 10.1136/ard.42.5.505. [DOI] [PMC free article] [PubMed] [Google Scholar]
  95. Zervas J., Vayopoulos G., Sakellaropoulos N., Kaklamanis P., Fessas P. HLA antigens and Adamantiades-Behçet's disease (A-BD) in Greeks. Clin Exp Rheumatol. 1988 Jul-Sep;6(3):277–280. [PubMed] [Google Scholar]
  96. Zouboulis C. C., Kötter I., Djawari D., Kirch W., Kohl P. K., Ochsendorf F. R., Keitel W., Stadler R., Wollina U., Proksch E. Epidemiological features of Adamantiades-Behçet's disease in Germany and in Europe. Yonsei Med J. 1997 Dec;38(6):411–422. doi: 10.3349/ymj.1997.38.6.411. [DOI] [PubMed] [Google Scholar]

Articles from Postgraduate Medical Journal are provided here courtesy of BMJ Publishing Group

RESOURCES