Bilateral Central Retinal Artery Occlusion in Behçet Disease

Abstract

Purpose: To report a case of bilateral central retinal artery occlusion as the primary ocular manifestation of Behçet disease.

Methods: A 38-year-old patient, with no history of previous ocular complaints, admitted with bilateral sudden vision loss. His ophthalmologic examination revealed a visual acuity of no light perception in both eyes and the diagnosis of bilateral central retinal artery occlusion. A detailed systemic investigation was conducted.

Results: With the history of recurrent oral and genital ulcers, and the presence of ocular and skin findings, the diagnosis of Behçet disease was made. Despite a potent immunosuppressive therapy the patient became absolutely blind.

Conclusions: Even though retinal arteries are rarely affected in Behçet disease, in the cases of retinal artery occlusion, it should be considered among the differential diagnosis. To our knowledge this is the first reported case of bilateral central retinal artery occlusion as the primary ocular manifestation of Behçet disease.     

Rapid and Sustained Efficacy of Golimumab in the Treatment of Multirefractory Uveitis Associated with Behçet’s Disease

Purpose: To evaluate golimumab (GOL) efficacy in the management of Behçet’s disease (BD)-related uveitis.

Methods: We retrospectively collected data from 5 patients (8 eyes) with at least two recent relapses of uveitis, treated with GOL at the standard dose of 50 mg every 4 weeks.

Results: A complete control of intraocular inflammation was observed in 7/8 eyes (87.5%) at 12-month follow-up. The number of relapses 12 months before and after GOL initiation was 11 and 1, respectively. At baseline, four eyes had active retinal vasculitis (RV). At 3-month follow-up evaluation RV resolved in all eyes. Mean Best Corrected Visual Acuity was 6.93 ± 4.34 at baseline and 7.32 ± 3.87 at 12-months follow-up.

Conclusion: We confirm GOL efficacy in reducing intraocular inflammation in BD, both in term of reduction in the number of uveitis relapses and in achieving a prompt resolution of active RV.     

Safety and Efficacy of Gevokizumab in Patients with Behçet’s Disease Uveitis: Results of an Exploratory Phase 2 Study

Purpose: To evaluate the safety and efficacy of gevokizumab for the treatment of Behçet’s disease uveitis in a prospective, open-label, randomized phase 2 trial.

Methods: Behçet’s disease patients with new acute ocular exacerbation or at risk of exacerbation received 30 or 60 mg gevokizumab every 4 weeks intravenously or subcutaneously, on top of a stable regimen of immunosuppressives and corticosteroids (≤20 mg/day equivalent prednisolone). Patients withdrew in cases of ocular exacerbation.

Results: A total of 21 patients were included (17 acute and 4 at-risk; mean duration of uveitis 45.6 ± 37.4 months). There were no serious adverse events related to gevokizumab. Recorded adverse events were mostly associated with exacerbation of uveitis or its complications. Response was evaluated for 14 acute patients and all showed rapid control of acute ocular exacerbation, mostly within 1 week, without any increase in corticosteroid dosage.

Conclusions: Gevokizumab was well tolerated and rapidly controlled acute ocular exacerbations of Behçet’s disease uveitis without the need for high-dose corticosteroid.     

Pattern of Retinal Vasculitis in an Egyptian Cohort

Purpose: To describe the clinical experience with retinal vasculitis at 4 Egyptian tertiary eye centers.

Methods: A multicenter retrospective chart review of all patients with retinal vasculitis encountered at 4 ocular inflammation referral clinics in Egypt between February 2013 and February 2018.

Results: The study included 618 patients (327 males and 291 females). Of these, 284 patients had isolated retinal vasculitis, whereas 233 patients had an associated systemic inflammatory disease, the most frequent being Behçet’s disease, followed by sarcoidosis. In 101 patients, retinal vasculitis could be attributed to an infectious etiology, and among this category, the most common was tuberculosis, followed by toxoplasmosis.

Conclusion: In our Egyptian cohort, more than half the patients had their retinal vasculitis as part of a serious systemic disease, or as part of an infectious process, as tuberculosis. And in view of the significant potential morbidity of these conditions, the local ophthalmologists need to be aware of these entities when faced with patients presenting with retinal vasculitis.     

Inflammatory bowel disease (Crohn's disease) in a Spanish patient with pars plana exudates: Report of a new case and review of the literature

Several ocular manifestations have been found in Crohn's disease patients, most often affecting the anterior segment. This paper presents the case of a young woman with pars plana exudates in whom Crohn's disease was later diagnosed. To the authors' knowledge, this is only the second report of Crohn's disease and concomitant pars plana exudates.     

Patterns of Uveitis in Patients Admitted to a University Hospital in Riyadh,Saudi Arabia

Purpose: To analyze clinical patterns, causes, and systemic disease associations among patients with uveitis admitted to King Abdulaziz University Hospital.

Methods: The authors retrospectively reviewed the medical records of 351 patients (600 eyes).

Results: The study subjects consisted of 183 (52.1%) males and 168 (47.9%) females, with a mean age of 39.9 ± 14.2 years at presentation. The most common anatomic diagnosis was panuveitis (68.6%), followed by posterior uveitis (12.7%), anterior uveitis (12.7%), and intermediate uveitis (6.0%). The most common identifiable specific diagnoses were presumed tuberculous uveitis (PTU) (28.2%), Vogt-Koyanagi-Harada (VKH) disease (19.4%), Behçet disease (BD) (12.5%), and toxoplasmosis (8.2%). After a mean follow-up period of 29.5 ± 22.1 months, 63.5% of the eyes achieved visual acuity of 20/40 or better. Eyes from patients with PTU had the worst final visual outcome.

Conclusions: The most common anatomic diagnosis was panuveitis. PTU, VKH disease, BD, and toxoplasmosis were the most frequent specific diagnoses.     

Patterns of Uveitis in a Tertiary Care Referral Institute in Saudi Arabia

Purpose: To present the pattern, causes and complications of uveitis in one of the tertiary eye centers in Saudi Arabia.

Methods: In this retrospective study, clinical records of randomly selected cases of uveitis attending the King Khaled Eye Specialist Hospital, Saudi Arabia, from 2001 to 2010, were reviewed.

Results: Our series included 888 out of 1286 cases of uveitis. Among them, there were 390 men (43.9%) with a mean age of 39.6 ± 11 (range: 6–94 years). The prevalence of uveitis among all eye patients was 1.5% and the annual incidence was 129 cases. Uveitis was bilateral in 567 patients (63.8%), mainly anterior in 27.3%, intermediate in 12.7%, posterior in 7.1%, while 52.9% were panuveitis. Infective etiology was seen in 180 patients (20.3%), of which presumed tuberculous uveitis (PTU) was the commonest cause in 94 patients (52%), followed by herpetic in 46 (26%) and toxoplasmosis in 29 (16%). Vogt–Koyanagi–Harada (VKH) 22%, Behçet uveitis (BU) 14.6%, idiopathic anterior uveitis 12.5%, presumed tuberculous uveitis (PTU) 10.6%, and idiopathic intermediate uveitis (10.1%) accounted for the majority of cases, whereas other diagnostic entities accounted for 30%. The most common cause of anterior uveitis was idiopathic uveitis (101; 41.7%), followed by herpetic (46; 19%). Toxoplasmosis was the most frequent cause of posterior uveitis (29; 46.3%). Intermediate uveitis was most commonly idiopathic (86; 76.1%). VKH disease was the most common cause of panuveitis (195; 41.5%), followed by Behçet disease (118; 25.1%). The common complications of uveitis were glaucoma (34.1%), posterior synechiae (22.3%) and cataract (15.1%).

Conclusions: The most common anatomic diagnosis was panuveitis. VKH disease and Behçet disease were the most frequent causes and glaucoma was the most common complication of uveitis.     

Susac syndrome: microangiopathy of the retina, cochlea and brain

Background : Susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. Methods : Analysis of two male and two female cases of Susac syndrome recognized in Australia. Results : In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleovestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of Susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behçet’s syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro‐otologic features are most frequently misdiagnosed as multiple sclerosis. Conclusion : Susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.     

Ocular Manifestations and Visual Outcomes of Behçet’s Uveitis in a Thai population

Purpose: To report on ocular manifestations and visual outcomes of Thai patients with Behçet’s Uveitis (BU).

Methods: We reviewed medical records of 50 BU patients (31 males and 19 females). Ocular manifestations, treatment modalities, complications, and visual outcomes were registered.

Results: Ocular involvement was bilateral in 76% of patients, resulting in 89 affected eyes. Panuveitis and posterior uveitis were the most common types. Retinal vasculitis was noted in majority of affected eyes and specifically arteritis was noticed in 32/57(56%). Most patients received combination therapy of systemic corticosteroids and immunosuppressive agents (azathioprine 72%). At final visit, VA ≤20/200 was observed in 25 affected eyes (28%). Risk factors for poor visual outcome were poor visual acuity at presentation (p < 0.001) and development of optic atrophy (p = 0.01).

Conclusions: Typical ocular manifestations of Thai patients with BU consisted of bilateral uveitis affecting posterior eye segment with high rate of complications and frequent visual loss.     

Clinical Patterns and Causes of Uveitis in a Referral Eye Clinic in Qatar

Purpose: To determine the patterns and causes of uveitis in a referral eye clinic in Qatar.

Methods: A retrospective study was conducted on all cases of uveitis that presented to the Uveitis Clinic at Hamad Medical Corporation from March 2007 to February 2011. All patients had detailed ocular, medical examination, and uveitis work up.

Results: The study included 310 patients. Anterior uveitis was noted in 53.2% of cases (165/310), followed by panuveitis 16.8% (52/310), intermediate uveitis 16.5% (51/310), and posterior uveitis 13.6% (42/310). Causes of uveitis were determined in 69.7% (216/310) of cases. The most commonly identified causes of uveitis in our study were presumed ocular tuberculosis (14.5%), Fuchs uveitis (11.0%), Behçet disease (7.4%), and VKH disease (6.8%).

Conclusions: Anterior uveitis is the most common anatomic/clinical form of uveitis in Qatar and the etiologic diagnosis of uveitis should focus in particular on presumed ocular tuberculosis, Fuchs uveitis, Behçet disease, and VKH disease.     

Pattern of Uveitis in a Referral Ophthalmology Center in the Central District of Thailand

Abstract

Purpose: To report the pattern of uveitis in a major ophthalmology center in the central district of Thailand.

Methods: A retrospective study was performed in uveitis cases visiting the Department of Ophthalmology at Rajavithi Hospital, Thailand, from January 2007 to October 2012.

Results: Four hundred and forty-six patients (mean age 42 years, female 53.8%) were included in the study. Uveitis was unilateral in 51.1% of cases. Anterior uveitis was the most common (44.8%) case, closely followed by panuveitis (40%), posterior uveitis (14.3%), and intermediate uveitis (0.9%). Specific diagnosis was established in 51.6% of patients. The three most common specific diagnoses were Vogt–Koyanagi-Harada (VKH) disease (22.4%), followed by Behçet disease (6.7%) and herpetic anterior uveitis (5.8%).

Conclusions: The most common type of noninfectious uveitis group was VKH, while herpetic anterior uveitis was the most common type of infectious uveitis in the central district of Thailand.     

Ten-Year Retention Rate of Infliximab in Patients with Behçet’s Disease-Related Uveitis

Purpose: To evaluate the 10-year drug retention rate of infliximab (IFX) in Behçet’s disease (BD)-related uveitis, the effect of a concomitant use of disease modifying anti-rheumatic drugs (DMARDs) on drug survival and differences according to the lines of biologic treatment.

Methods: Cumulative survival rates were studied using the Kaplan-Meier plot, while the Log-rank (Mantel-Cox) test was used to compare survival curves.

Results: Forty patients (70 eyes) were eligible for analysis. The drug retention rates at 12-, 24-, 60- and 120-month follow-up were 89.03%, 86.16%, 75.66% and 47.11% respectively. No differences were identified according to the use of concomitant DMARDs (p = 0.20), while a statistically significant difference was observed in relation to the different lines of IFX treatment (p = 0.014). Visual acuity improved from baseline to the last follow-up visit (p = 0.047) and a corticosteroid-sparing effect was observed (p < 0.0001).

Conclusions: IFX retention rate in BD-uveitis is excellent and is not affected by concomitant DMARDs.     

视网膜分支动脉阻塞视力预后相关因素分析

目的：研究视网膜分支动脉阻塞(branch retinal artery occlusion,BRAO)患眼视力预后的相关因素分析。

方法：选取2015-06/2017-06来我院就诊并诊断为视网膜分支动脉阻塞的患者68例。收集患者的年龄、性别、发病时间等基本信息及初诊时最佳矫正视力、眼底彩色照相、频域OCT及OCTA等眼科检查结果。OCTA检查采用RTVue XRAVANTI仪进行。选择视网膜血流成像扫描模式,扫描区域分别为黄斑区3mm×3mm。随访时间为6mo,随访时检查最佳矫正视力。

结果：患者就诊时基线视力：轻度视力损害(≥0.5)为69%,中度视力损害(0.1～0.4)为24%,重度视力损害(<0.1)为7%; 随访预后视力的轻、中、重度损害分别为75%、19%、6%。影响预后视力的相关因素分析：就诊时基线视力(r=0.776,P<0.001)、浅层视网膜毛细血管黄斑区拱环存留度(r=-0.003,P<0.001)、深层视网膜毛细血管黄斑区拱环存留度(r=-0.003,P<0.001)、黄斑区浅层毛细血管密度(r=-0.034,P=0.029)及黄斑区深层毛细血管密度(r=-0.014,P=0.012)与预后视力密切相关。性别、年龄、就诊时间、黄斑中心凹厚度、视网膜动脉受阻位置(颞上或颞下)以及是否发现明确视网膜血管动脉栓子与预后视力无相关性(r=-0.273、0.01、0、0.82、0.41、0.109,均P>0.05)。

结论：BRAO患眼黄斑拱环存留度、黄斑区毛细血管密度和发病早期视力与预后视力密切相关。     

Binasal hemianopias from presumed intrageniculate myelinolysis: report of a case with MR images of bilateral lateral geniculate involvement after emergency cesarean section and hysterectomy

A 33-year-old woman developed dense bilateral nasal hemianopias after near-fatal uterine hemorrhage and hysterectomy. Magnetic resonance imaging of the brain showed unique hyperintense lateral geniculate lesions presumed to represent osmotic demyelination from extrapontine myelinolysis.     

Acute visual loss with ophthalmoplegia after spinal surgery: Report of a case and review of the literature

We report a case of a 15-year-old boy who presented with profound visual loss and complete ophthalmoplegia after an uneventful spinal surgery for removal of cervical osteoblastoma. Postoperative visual loss following nonocular surgery is, fortunately rare, yet a devastating complication. The most common cause is ischemic optic neuropathy, but it can also be due to central retinal occlusion or cortical blindness. Visual loss in conjunction with ophthalmoplegia is rarely seen, and there are very few reports in the literature. We also review the related literature and highlight the mechanism and preventive measures.     

Detection of Mycobacterium tuberculosis by polymerase chain reaction in a case of orbital tuberculosis

Orbital tuberculosis is quite uncommon. We report a case of orbital tuberculosis in a 3-year-old child from Bangladesh who presented with swelling and discharging sinus in the lower part of the orbit. Histopathology revealed a granulomatous inflammation with caseation necrosis. Polymerase chain reaction (PCR) showed amplification of the Mycobacterium tuberculosis genome. The patient responded to a course of antituberculous treatment. Mycobacterium tuberculosis should be considered in the differential diagnosis of inflammatory orbital disease in the Indian subcontinent where tuberculosis is prevalent.     

Detection of Toxoplasma gondii in aqueous humour by the polymerase chain reaction.

Ocular toxoplasmosis is the most frequent infectious cause of chorioretinal inflammation in immunocompetent patients. Nowadays, the biological diagnosis of ocular toxoplasmosis requires serological tests and anterior chamber puncture to detect the local production of specific antibodies. A new technique is described to detect Toxoplasma in aqueous humour by a polymerase chain reaction in which the target is a specific ribosomal DNA segment. Sixty eight patients (71 eyes) were included; 59 (83%) eyes were suspected of having ocular toxoplasmosis. Of these 59 eyes, 15 (25.4%) had characteristic fundus lesions with obvious intraocular inflammation signs and 44 (75%) had retinal scar of ocular toxoplasmosis without clinically detectable inflammation. Twelve (17%) eyes had uveitis of non-Toxoplasma origin and constituted the control group. The parasite was present in aqueous humour in 20 (33.8%) cases. No false positives were detected. The sensitivity of the test is reduced by the low numbers of the sample. The combination of this technique with Witmer-Desmonts coefficient increases the probability of making a biological diagnosis of ocular toxoplasmosis. The physiopathological value of this technique is emphasised and the presence of tachyzoites in the anterior chamber is suggested. This should be a very promising technique for the diagnosis of ocular toxoplasmosis.