A 68-year-old fair-skinned Indian woman presented to our hospital with a primary complaint of vision loss in her right eye, accompanied by increased light sensitivity, pain, and redness that had persisted for one month. Additionally, she reported progressive visual impairment in her left eye over the past two years, along with oral and genital lesions, and multiple joint pains, for which she had been receiving ongoing treatment. She had previously been diagnosed with Behçet's uveitis, with systemic involvement, according to the criteria outlined by an international study group on Behçet's disease. On examination, the right eye showed pan-uveitis with a large optic disc cup and significant disc pallor. The left eye had corneal decompensation and was phthisical. Fundus fluorescein angiography (FFA) detected minimal disc leakage and peripheral vascular leaks. Optical coherence tomography angiography (OCT-A) revealed increased retinal nerve fibre layer loss, large cup, and severe peri-papillary capillary dropout. Macular OCT-A showed significant capillary loss, widened inter-capillary distances, and an enlarged foveal avascular zone. Laboratory tests revealed a strongly positive antinuclear antibody (ANA) with a speckled homogenous pattern. The erythrocyte sedimentation rate (ESR) was elevated at 52 mm/hr, and C-reactive protein (CRP) was 6.12 mg/dl. The differential leukocyte count showed elevated segmented neutrophils, while other blood parameters were normal. Serology for tuberculosis (TB), HLA-B27, p-ANCA, c-ANCA, and anti-CCP was negative. Based on her clinical presentation, histopathology, and investigations, she was diagnosed with Behçet's eye disease with multi-system involvement, including gastrointestinal and joint disease, and confirmed to have right-eye ischemic optic neuropathy. She was treated with topical steroids and intravenous methylprednisolone, resulting in significant visual improvement. After eight weeks, her vision improved to 6/36, with better contrast and color vision. Mycophenolate mofetil, oral steroids, and tacrolimus were restarted. Due to poor response to oral medications, intravenous adalimumab was considered but discontinued due to side effects. At three months, her right-eye vision stabilized at 6/24, with normal color perception, improved contrast sensitivity, and stable lab results.
Published in | International Journal of Ophthalmology & Visual Science (Volume 10, Issue 1) |
DOI | 10.11648/j.ijovs.20251001.12 |
Page(s) | 16-22 |
Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
Copyright |
Copyright © The Author(s), 2025. Published by Science Publishing Group |
AION, Behcets, Uveitis, OCT-A
[1] | Zafirakis P, Foster Stephen C. (2013). Adamantiades-Bechets Disease. Chapter 58. Pages 858-886. Diagnosis and treatment of uveitis. 2nd edition. C Stephen foster and Albert T Vitale. Jaypee Highlights Medical Publishers, Inc. |
[2] | Ohno S. Bechets disease in the world. In: Lehmer T, Barne CG, (Eds). Recent advances in Bechets disease, London: Royal society of Medicine Services: 1996. |
[3] | Chajek T, Fainaru M. Behçet's disease. Report of 41 cases and a review of the literature. Medicine (Baltimore). 1975 May; 54(3): 179-96. |
[4] | Stratigos AJ, Laskaris G, Stratigos JD. Behçet's disease. Semin Neurol. 1992 Dec; 12(4): 346-57. |
[5] | O Duffy JD. Suggested criteria for diagnosis of Bechets disease. J Rheumatol. 1974: 1(suppl): 18. |
[6] | Hayreh SS. Blood supply of the optic nerve head. Ophthalmologica. 1996; 210(5): 285-95. |
[7] | Tong B, Liu X, Xiao J, Su G. Immunopathogenesis of Behcet's Disease. Front. Immunol. 2019; 10: 665. |
[8] | Frigui M, Kechaou M, Jemal M, Ben Zina Z, Feki J, Bahloul Z. Les neuropathies optiques au cours de la maladie de Behçet: à propos de 18 cas. Rev Med Interne. 2009 Jun; 30(6): 486-91. French. |
[9] | Zhu LP, Lai CT, Yang QL, Li Z, Shi XH, Meng C, Wang JW. The clinical analysis of optic neuropathy relative to Behcet's disease with clinical manifestation of anterior ischemic optic neuropathy]. Zhonghua Yan Ke Za Zhi. 2019 Mar 11; 55(3): 203-207. Chinese. |
[10] | Ozdal PC, Ortaç S, Taşkintuna I, Firat E. Posterior segment involvement in ocular Behçet's disease. Eur. J. Ophthalmol. 2002; 12: 424–431. |
[11] | Yan C, Li F, Hou M, Ye X, Su L, Hu Y, Luo J, Chi W. Vascular Abnormalities in Peripapillary and Macular Regions of Behcet's Uveitis Patients Evaluated by Optical Coherence Tomography Angiography. Front Med (Lausanne). 2021 Sep 16; 8: 727151. |
[12] | Küçük MF, Yaprak L, Erol MK, Ayan A, Kök M. Quantitative changes in peripapillary, macular, and choriocapillaris microvasculature of patients with non-ocular Behçet's disease and relationship with systemic vascular involvement, an optical coherence tomography angiography study. Photodiagnosis Photodyn Ther. 2022 Jun; 38: 102749. |
[13] | Simsek M, Aksoy M, Ulucakoy RK. Evaluation of retinal and choroidal microcirculation in Behçet's disease. Eye (Lond). 2022 Jul; 36(7): 1494-1499. |
APA Style
Arora, D., Sharma, A., Sharma, D., Sharma, D. (2025). Multimodal Imaging in a Case of Behcets Uveitis with Anterior Ischemic Optic Neuropathy: A Microvascular Perspective. International Journal of Ophthalmology & Visual Science, 10(1), 16-22. https://doi.org/10.11648/j.ijovs.20251001.12
ACS Style
Arora, D.; Sharma, A.; Sharma, D.; Sharma, D. Multimodal Imaging in a Case of Behcets Uveitis with Anterior Ischemic Optic Neuropathy: A Microvascular Perspective. Int. J. Ophthalmol. Vis. Sci. 2025, 10(1), 16-22. doi: 10.11648/j.ijovs.20251001.12
@article{10.11648/j.ijovs.20251001.12, author = {Deepesh Arora and Anuj Sharma and Devesh Sharma and Dinesh Sharma}, title = {Multimodal Imaging in a Case of Behcets Uveitis with Anterior Ischemic Optic Neuropathy: A Microvascular Perspective }, journal = {International Journal of Ophthalmology & Visual Science}, volume = {10}, number = {1}, pages = {16-22}, doi = {10.11648/j.ijovs.20251001.12}, url = {https://doi.org/10.11648/j.ijovs.20251001.12}, eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijovs.20251001.12}, abstract = {A 68-year-old fair-skinned Indian woman presented to our hospital with a primary complaint of vision loss in her right eye, accompanied by increased light sensitivity, pain, and redness that had persisted for one month. Additionally, she reported progressive visual impairment in her left eye over the past two years, along with oral and genital lesions, and multiple joint pains, for which she had been receiving ongoing treatment. She had previously been diagnosed with Behçet's uveitis, with systemic involvement, according to the criteria outlined by an international study group on Behçet's disease. On examination, the right eye showed pan-uveitis with a large optic disc cup and significant disc pallor. The left eye had corneal decompensation and was phthisical. Fundus fluorescein angiography (FFA) detected minimal disc leakage and peripheral vascular leaks. Optical coherence tomography angiography (OCT-A) revealed increased retinal nerve fibre layer loss, large cup, and severe peri-papillary capillary dropout. Macular OCT-A showed significant capillary loss, widened inter-capillary distances, and an enlarged foveal avascular zone. Laboratory tests revealed a strongly positive antinuclear antibody (ANA) with a speckled homogenous pattern. The erythrocyte sedimentation rate (ESR) was elevated at 52 mm/hr, and C-reactive protein (CRP) was 6.12 mg/dl. The differential leukocyte count showed elevated segmented neutrophils, while other blood parameters were normal. Serology for tuberculosis (TB), HLA-B27, p-ANCA, c-ANCA, and anti-CCP was negative. Based on her clinical presentation, histopathology, and investigations, she was diagnosed with Behçet's eye disease with multi-system involvement, including gastrointestinal and joint disease, and confirmed to have right-eye ischemic optic neuropathy. She was treated with topical steroids and intravenous methylprednisolone, resulting in significant visual improvement. After eight weeks, her vision improved to 6/36, with better contrast and color vision. Mycophenolate mofetil, oral steroids, and tacrolimus were restarted. Due to poor response to oral medications, intravenous adalimumab was considered but discontinued due to side effects. At three months, her right-eye vision stabilized at 6/24, with normal color perception, improved contrast sensitivity, and stable lab results. }, year = {2025} }
TY - JOUR T1 - Multimodal Imaging in a Case of Behcets Uveitis with Anterior Ischemic Optic Neuropathy: A Microvascular Perspective AU - Deepesh Arora AU - Anuj Sharma AU - Devesh Sharma AU - Dinesh Sharma Y1 - 2025/03/07 PY - 2025 N1 - https://doi.org/10.11648/j.ijovs.20251001.12 DO - 10.11648/j.ijovs.20251001.12 T2 - International Journal of Ophthalmology & Visual Science JF - International Journal of Ophthalmology & Visual Science JO - International Journal of Ophthalmology & Visual Science SP - 16 EP - 22 PB - Science Publishing Group SN - 2637-3858 UR - https://doi.org/10.11648/j.ijovs.20251001.12 AB - A 68-year-old fair-skinned Indian woman presented to our hospital with a primary complaint of vision loss in her right eye, accompanied by increased light sensitivity, pain, and redness that had persisted for one month. Additionally, she reported progressive visual impairment in her left eye over the past two years, along with oral and genital lesions, and multiple joint pains, for which she had been receiving ongoing treatment. She had previously been diagnosed with Behçet's uveitis, with systemic involvement, according to the criteria outlined by an international study group on Behçet's disease. On examination, the right eye showed pan-uveitis with a large optic disc cup and significant disc pallor. The left eye had corneal decompensation and was phthisical. Fundus fluorescein angiography (FFA) detected minimal disc leakage and peripheral vascular leaks. Optical coherence tomography angiography (OCT-A) revealed increased retinal nerve fibre layer loss, large cup, and severe peri-papillary capillary dropout. Macular OCT-A showed significant capillary loss, widened inter-capillary distances, and an enlarged foveal avascular zone. Laboratory tests revealed a strongly positive antinuclear antibody (ANA) with a speckled homogenous pattern. The erythrocyte sedimentation rate (ESR) was elevated at 52 mm/hr, and C-reactive protein (CRP) was 6.12 mg/dl. The differential leukocyte count showed elevated segmented neutrophils, while other blood parameters were normal. Serology for tuberculosis (TB), HLA-B27, p-ANCA, c-ANCA, and anti-CCP was negative. Based on her clinical presentation, histopathology, and investigations, she was diagnosed with Behçet's eye disease with multi-system involvement, including gastrointestinal and joint disease, and confirmed to have right-eye ischemic optic neuropathy. She was treated with topical steroids and intravenous methylprednisolone, resulting in significant visual improvement. After eight weeks, her vision improved to 6/36, with better contrast and color vision. Mycophenolate mofetil, oral steroids, and tacrolimus were restarted. Due to poor response to oral medications, intravenous adalimumab was considered but discontinued due to side effects. At three months, her right-eye vision stabilized at 6/24, with normal color perception, improved contrast sensitivity, and stable lab results. VL - 10 IS - 1 ER -