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
AION | Anterior Ischemic Optic Neuropathy |
ANA | Antinuclear Antibody |
Anti-CCP | Anti-cyclic Citrullinated Peptide |
BD | Adamantiades Behcets Disease |
BCVA | Best Corrected Visual Acuity |
CRP | C-reactive Protein |
ESR | Erythrocyte Sedimentation Rate |
FFA | Fundus Fluorescein Angiography |
IOP | Intraocular Pressure |
HLA B27 | Human Leucocyte Antigen |
MMV | Macular Microvascular Architecture |
OCT | Optical Coherence Tomography |
OCT-A | Optical Coherence Tomography Angiography |
RNFL | Retinal Nerve Fibre Layer |
RPC | Radial Peripapillary Capillaries |
TB | Tuberculosis |
pANCA | Perinuclear Anti-Neutrophil Cytoplasmic Antibody |
cANCA | Cytoplasmic Anti-Neutrophil Cytoplasmic Antibody |
CNS | Central Nervous System |
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APA Style
Arora, D., Sharma, A., Sharma, D., Sharma, D. (2025). Multimodal Imaging in a Case of Behcets Uveitis with Anterior Ischcmic 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 Ischcmic Optic Neuropathy: A Microvascular Perspective. Int. J. Ophthalmol. Vis. Sci. 2025, 10(1), 16-22. doi: 10.11648/j.ijovs.20251001.12
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Download@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 Ischcmic 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 Ischcmic 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 -
Department of Vitreo-Retina and Uvea, Amritsar Eye Clinic, Dehradun, India
Department of Vitreo-Retina and Uvea, Amritsar Eye Clinic, Dehradun, India
Department of Cornea, Cataract and Refractive Surgery, Amritsar Eye Clinic, Dehradun, India
Department of Cornea, Cataract and Refractive Surgery, Amritsar Eye Clinic, Dehradun, India
Figure 1. Colour fundus photo shows disc pallor with horizontal cup disc ratio at 0.7:1.
Figure 2. Colour fundus photo 1 year apart from (Figure 1) shows gross disc pallor with horizontal cup disc ratio at 0.9:1 with distinct peripapillary atrophy not seen in (Figure 1) with marked tessellation.
Figure 3. FFA shows minimal vascular leaks from disc and leakage seen in periphery near oral bay.
Figure 4. FFA one year apart from (Figure 3) shows staining of peripapillary atrophy with loss of macula vasculature with no leak detected in comparison to (Figure 3) suggesting quiesant phase of Behcets uveitis.
Figure 5. Angiovue Disc shows a right eye large cup with RNFL thickness loss in all quadrants most significant in temporal quadrant. Radial peripapillary capillary vessel density (%) of small vessels depicts the overall vascular loss (Depicted in blue as area of loss and maintained vessel depicted in brown) in coloured disc photo.
Figure 6. Angiovue Disc right eye one year apart from (Figure 5) shows a large cup with RNFL thickness loss in all quadrants most significant in temporal quadrant. Radial peripapillary capillary vessel density (%) of small vessels depicts the overall one year vascular loss compared to (Figure 5) to be much larger, suggesting the ongoing process of ischemia in Behcets uveitis (Depicted in blue as area of radial peripapillary vascular loss).
Figure 7. Angiovue 3 mm quickvue scan of right macula shows changes in microvasculature most prominent in superficial and deep capillary plexus with poor visibility of outer retina and choriocapillaries suggesting active inflammation obscuring penetration. There is distinct widening of foveal avascular zone, loss of major capillaries with widening of intercapillary distance.
Figure 8. Angiovue retina quickvue shows a major decline in capillaries in superficial and deep plexus in to comparison to (Figure 7). There is also loss of foveal plexus margin with complete loss of intercapillary plexus. There is loss seen in outer retina and choriocapillaries primarily in inferior half suggesting major site of effect of Behcets uveitis.
Figure 9. OCT macula radial scan shows multi pocket neurosensory detachment, inner retinal odema, subretinal fluid and increase in retinochoroidal thickness with loss of RPE and IS-OS junction details.
Figure 10. OCT macula radial scan 4 months apart from (Figure 9) shows streak of sub-retinal fluid with loss of inner retinal oedema with multiple hyper-reflective dots seen in inner layers suggesting a quiesant phase with undulations of retinochoroidal complex.
Figure 11. Behcets patient with eruptions and ulcers on tongue.
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