The aim of this study was to analyze ocular manifestations, their complications, and treatment in a sample of 40 patients with confirmed Behcet’s disease. Results. Serofibrinous iridocyclitis was the most common form of uveitis (60%). Retinal periphlebitis manifested in 92.5% of cases, and periphlebitis in conjunction with periarteritis was diagnosed in 72.5% of cases. Macular edema was the most frequent complication on the posterior segment (60%) and it correlated with periphlebitis ( ) and periphlebitis associated with periarteritis ( ). Cyclosporine A and corticosteroids were used in the majority of cases (67%). Following six months of therapy, a significant improvement of visual acuity occurred in patients with initial visual acuity >0.5 on both eyes. Level of visual acuity before and after treatment had a strong significant correlation coefficient with various ocular complications. Previously proven significant increase of visual acuity in patients with macular edema depicts effectiveness of treatment in these types of ocular manifestations of Behcet’s disease. Conclusions. Significant improvement of visual acuity occurred in patients with initial visual acuity >0.5 on both eyes. The highest increase in visual acuity was achieved by laser photocoagulation in combination with triamcinolone acetonide . 1. Introduction Behcet’s disease is anidiopathic, multisystem inflammatory chronic relapsing disorder, systemic occlusive vasculitis, which affects veins and arteries of all sizes. Disease is characterized by episodic inflammation which may affect every tissue and organ in the body [1–6]. International Study Group for Behcet’s Disease has established diagnostic criteria: ocular lesions, oral aphthosis, and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement, and vascular manifestations 1 point each. The pathergy test, when used, was assigned 1 point. A patient scoring ≥4 points is classified as having Behcet’s disease [7]. Behcet’s disease has been associated with HLA-B51 phenotype, but the strength of this association varies worldwide [8–18]. Ocular features of Behcet’s disease manifest as anterior uveitis and retinal vasculitis (both veins and arteries) which result in progressive, irreversible, ischemic damage of the retina and optic nerve. Ocular involvement occurs in approximately 70% of patients and is associated with high risk of blindness [19–22]. Cataract, secondary glaucoma, optic disc atrophy, and macular edema are the most common complications in Behcet’s disease leading to decrease of vision
References
[1]
G. Hatemi, E. Seyahi, I. Fresko, and V. Hamuryudan, “Behcet’s syndrome: a critical digest of the recent literature,” Clinical and Experimental Rheumatology, vol. 30, no. 3, supplement 72, 2012.
[2]
P. McCluskey and P. R. J. Powell, “The eye in systemic inflammatory diseases,” Lancet, vol. 364, no. 9451, pp. 2125–2133, 2004.
[3]
C. Evereklioglu, “Managing the symptoms of Behcet’s disease,” Expert Opinion on Pharmacotherapy, vol. 5, pp. 317–328, 2004.
[4]
F. Davatchi, F. Shahram, C. Chams, H. Chams, and A. Nadji, “Beh？et's disease,” Acta Medica Iranica, vol. 43, no. 4, pp. 233–242, 2005.
[5]
N. Mizuki, M. Ota, and C. Katsuyama Everekioglu, “Current concepts in the etiology and treatment of disease,” Survey of Ophthalmology, vol. 50, article 4, 2005.
[6]
I. Krause and A. Weinberger, “Beh？et's disease,” Current Opinion in Rheumatology, vol. 20, no. 1, pp. 82–87, 2008.
[7]
International Team for the Revision of the International Criteria for Beh？et's Disease (ITR-ICBD), “The International Criteria for Beh？et's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria,” Journal of the European Academy of Dermatology and Venereology, 2013.
[8]
N. Mizuki, H. Inoko, and S. Ohno, “Pathogenic gene responsible for the predisposition to Behcet's disease,” International Reviews of Immunology, vol. 14, no. 1, pp. 33–48, 1997.
[9]
K. Yabuki, H. Ando, T. Shiina, et al., “HLA-B*51 allele analysis by the PCR-SBT method and a strong association of HLA-B*5101 with Japanese patients with Behcet’s disease,” Tissue Antigens, vol. 58, pp. 181–184, 2001.
[10]
T. ？rfan Kaya, “Genetics of Beh？et's disease,” Pathology Research International, vol. 2012, Article ID 912589, 6 pages, 2012.
[11]
D. J. Kilmartin, A. Finch, and R. W. Acheson, “Primary association of HLA-B51 with Beh？et's disease in Ireland,” British Journal of Ophthalmology, vol. 81, no. 8, pp. 649–653, 1997.
[12]
M. De Menthon, M. P. LaValley, C. Maldini, L. Guillevin, and A. Mahr, “HLA-B51/B5 and the risk of Beh？et's disease: a systematic review and meta-analysis of case-control genetic association studies,” Arthritis Care and Research, vol. 61, no. 10, pp. 1287–1296, 2009.
[13]
N. Mizuki and S. Ohno, “Immunogenetic studies of Behcet’s disease,” Revue du Rhumatisme, no. 7-8, pp. 520–527, 1996.
[14]
N. Mizuki, M. Ota, Y. Katsuyama et al., “HLA-B*51 allele analysis by the PCR-SBT method and a strong association of HLA-B*5101 with Japanese patients with Beh？et's disease,” Tissue Antigens, vol. 58, no. 3, pp. 181–184, 2001.
[15]
S.-H. Park, K.-S. Park, Y.-I. Seo et al., “Association of MICA polymorphism with HLA-B51 and disease severity in Korean patients with Behcet's disease,” Journal of Korean Medical Science, vol. 17, no. 3, pp. 366–370, 2002.
[16]
H. Direskeneli, “Beh？et's disease: infectious aetiology, new autoantigens, and HLA-B51,” Annals of the Rheumatic Diseases, vol. 60, no. 11, pp. 996–1002, 2001.
[17]
A. Guel, “Behcet’s disease: an update on the pathogenesis,” Clinical and Experimental Rheumatology, vol. 19, supplement 24, pp. S6–S12, 2001.
[18]
N. Kitaichi, A. Miyazaki, M. R. Stanford, H. Chams, D. Iwata, and S. Ohno, “Ocular features of Beh？et's disease: an international collaborative study,” British Journal of Ophthalmology, vol. 91, no. 12, pp. 1579–1582, 2007.
[19]
M. Takeuchi, H. Hokama, R. Tsukahara et al., “Risk and prognostic factors of poor visual outcome in Behcet's disease with ocular involvement,” Graefe's Archive for Clinical and Experimental Ophthalmology, vol. 243, no. 11, pp. 1147–1152, 2005.
[20]
K. Durrani, M. Ahmed, and C. S. Foster, “Adamantiades-Behcet disease: diagnosis and current concepts in management of ocular manifestations,” Comprehensive Ophthalmology Update, vol. 8, no. 4, pp. 225–233, 2007.
[21]
C. Evereklioglu, “Current concepts in the etiology and treatment of Beh？et disease,” Survey of Ophthalmology, vol. 50, no. 4, pp. 297–350, 2005.
[22]
M. Dias Ljopis, M. Cervera, and J. l. Mnezo, “Cyclosporin A treatment of Behcet’s disease: a long-term study,” Current Eye Research, vol. 9, pp. 17–23, 1990.
[23]
N. Stübiger and U. Pleyer, “Typical and atypical ocular manifestations of Beh？et's disease,” Ophthalmologe, vol. 109, no. 6, pp. 558–562, 2012.
[24]
N. Unoki, K. Nishijima, M. Kita, R. Hayashi, and N. Yoshimura, “Structural changes of fovea during remission of Beh？et's disease as imaged by spectral domain optical coherence tomography,” Eye, vol. 24, no. 6, pp. 969–975, 2010.
[25]
H. Yazisi, H. Pazarli, C. G. Barnes, et al., “A controlled trial of azathioprine in Behcet’s syndrome,” New England Journal of Medicine, vol. 322, pp. 281–285, 1997.
[26]
V. Humuryudan, Y. Ozyazgan, N. Hizli, et al., “Azathioprine in Behcet’s syndrome,” Arthritis and Rheumatism, vol. 40, pp. 760–774, 1997.
[27]
A. J. Greenwood, M. R. Stanford, and E. M. Graham, “The role of azathioprine in the management of retinal vasculitis,” Eye, vol. 12, no. 5, pp. 783–788, 1998.
[28]
V. G. Kaklamani and P. G. Kaklamanis, “Treatment of beh？et's disease—an update,” Seminars in Arthritis and Rheumatism, vol. 30, no. 5, pp. 299–312, 2001.
[29]
C. Mat, S. Yurdakul, S. Uysal et al., “A double-blind trial of depot corticosteroids in Beh？et's syndrome,” Rheumatology, vol. 45, no. 3, pp. 348–352, 2006.
[30]
C. M. Deuter, M. Zierhut, D. Doycheva, and I. K？tter, “Treatment of Behcet’s disease yesterday and today,” Ophthalmologe, vol. 109, no. 6, pp. 568–574, 2012.
[31]
U. Pleyer, D. Hazirolan, S. Winterhalter, and N. Stubiger, “Behcet’s disease-ophthalmological and general aspects—part 2: therapy,” Ophthalmologe, vol. 110, no. 3, pp. 273–284, 2013.
[32]
P. P. Sfikakis, N. Markomichelakis, E. Alpsoy et al., “Anti-TNF therapy in the management of Beh？et's disease—review and basis for recommendations,” Rheumatology, vol. 46, no. 5, pp. 736–741, 2007.
[33]
S. Sugita, Y. Kawazoe, A. Imai, Y. Yamada, S. Horie, and M. Mochizuki, “Inhibition of Th17 differentiation by anti-TNF-alpha therapy in uveitis patients with Behcet's disease,” Arthritis Research & Therapy, vol. 14, article R99, 2012.
[34]
T. Sakai, H. Watanabe, K. Kuroyanagi et al., “Health- and vision-related quality of life in patients receiving infliximab therapy for Behcet uveitis,” British Journal of Ophthalmology, vol. 97, no. 3, pp. 338–342, 2013.
[35]
S. Furuta, Y. W. Chow, A. N. Chaudhry, and D. Jayne, “Switching of anti-TNF-α agents in Behcet’s disease,” Clinical and Experimental Rheumatology, vol. 30, no. 3, supplement 72, pp. S62–S68, 2012.
[36]
E. W. Lindstedt, G. S. Baarsma, R. W. A. M. Kuijpers, and P. M. Van Hagen, “Anti-TNF-α therapy for sight threatening uveitis,” British Journal of Ophthalmology, vol. 89, no. 5, pp. 533–536, 2005.
[37]
P. P. Sfikakis, P. H. Kaklamanis, A. Elezoglou et al., “Infliximab for recurrent sight threatening ocular inflammation in Adamantiades-Behcet disease,” Annals of Internal Medicine, vol. 140, no. 5, pp. 404–406, 2004.
[38]
M. Accorinti, M. P. Pirraglia, M. P. Paroli, R. Priori, F. Conti, and P. Pivetti-Pezzi, “Infliximab treatment for ocular and extraocular manifestations of Beh？et's disease,” Japanese Journal of Ophthalmology, vol. 51, no. 3, pp. 191–196, 2007.
[39]
P. P. Sfikakis, P. G. Theodossiadis, C. G. Katsiari, P. Kaklamanis, and N. N. Markomichelakis, “Effect of infliximab on sight-threatening panuveitis in Beh？et's disease,” Lancet, vol. 358, no. 9278, pp. 295–296, 2001.
[40]
K. Nakae, F. Masaki, T. Hashimoto, G. Inaba, M. Moshiziki, and T. Sakane, “Recent epidemiological features of Behcet’s Disease in Japan,” in Behcet’s Disease, P. Godeau and B. Wechsler, Eds., pp. 145–151, Elsevier Science Publishers B. V., 1993.
[41]
F. Davatachi, F. Sharam, F. Gharibdoost et al., “Clinical manifestations of Behcet’s Disease in Iran: Behcet’s Disease. Analysis of 2806 cases,” Arthritis and Rheumatism, no. S391, 1995.
[42]
A. Gürler, A. Boyvat, and ü. Türsen, “Clinical manifestations of Beh？et's disease: an analysis of 2147 patients,” Yonsei Medical Journal, vol. 38, no. 6, pp. 423–427, 1997.
[43]
C. C. Zouboulis, “Epidemiology of Adamantiades-Behcet’s disease,” Annals of Internal Medicine, vol. 150, pp. 488–498, 1999.
[44]
T. Sakane, M. Takeno, N. Suzuki, and G. Inaba, “Behcet's disease,” New England Journal of Medicine, vol. 341, no. 17, pp. 1284–1291, 1999.
[45]
M. Muhaya, S. Lightman, E. Ikeda, et al., “Behcet’s Disease in Japan and Britain; a comparative study,” Ocular Immunology and Inflammation, vol. 8, pp. 141–148, 2000.
[46]
U. Tursen, A. Gurler, and A. Boyvat, “Evaluation of clinical findings according to sex in 2313 Turkish patients with Beh？et's disease,” International Journal of Dermatology, vol. 42, no. 5, pp. 346–351, 2003.
[47]
I. Tugal-Tutkun, S. Onal, R. Altan-Yaycioglu, H. Huseyin Altunbas, and M. Urgancioglu, “Uveitis in Beh？et disease: an analysis of 880 patients,” American Journal of Ophthalmology, vol. 138, no. 3, pp. 373–380, 2004.
[48]
M. H. Houman, H. Neffati, A. Braham et al., “Beh？et's disease in Tunisia demographic, clinical and genetic aspects in 260 patients,” Clinical and Experimental Rheumatology, vol. 25, no. 4, supplement 45, pp. S58–S64, 2007.
[49]
N. Ohguro, K. H. Sonoda, M. Takeuchi, M. Matsumara, and M. Mochizuku, “The prospective multi-center epidemiologic survey of uveitis in Japan,” Japanese Journal of Ophthalmology, 2012.
[50]
T. Akpolat, Y. Ko？, I. Yeniay et al., “Familial Beh？et's disease,” The European Journal of Medicine, vol. 1, no. 7, pp. 391–395, 1992.
[51]
U. Pleyer, D. Hazirolan, S. Winterhalter, and N. Stübiger, “Beh？et's disease—ophthalmological and general aspects—part I: etiology, pathogenesis and diagnostics,” Ophthalmologe, vol. 109, no. 11, pp. 1129–1141, 2012 (German).
