Bilateral Eales’ Disease Managed With Vitrectomy and Anti-VEGF Therapy: A Case Report

Introduction

Eales’ disease is an idiopathic occlusive retinal vasculopathy with an inflammatory basis that typically affects healthy men aged 20 to 40 years [1]. Although first described by Henry Eales in the 19th century, its pathogenesis remains incompletely understood. Two principal hypotheses have been proposed. One implicates hypersensitivity to Mycobacterium tuberculosis antigens, suggesting an infectious trigger [2]; the other emphasizes autoimmune-mediated vasculitis, supported by reports of systemic inflammatory associations and favorable responses to corticosteroids and immunosuppressive therapy [3,4]. In clinical practice, these mechanisms likely interact, leading to periphlebitis, venous occlusion, retinal ischemia, and eventual proliferative neovascularization [5].

Due to its rarity in Europe and North America, diagnostic suspicion remains low; many cases are identified only at advanced stages [4,6–9]. Established treatments are phase specific and include corticosteroids, laser photocoagulation, and vitrectomy [8–10]. Anti–vascular endothelial growth factor (VEGF) agents, such as bevacizumab, have been used with variable outcomes, including concerns regarding inconsistent efficacy and potential tractional progression [11,12].

A key gap in the literature is the absence of data regarding use of faricimab, a bispecific antibody that targets both vascular endothelial growth factor A (VEGF-A) and angiopoietin-2 (Ang-2). Dual-pathway inhibition not only suppresses neovascularization but also promotes vascular stabilization, which may be particularly valuable in inflammatory retinal vasculopathies [13–16]. To our knowledge, no previous report has described the use of faricimab in Eales’ disease. Accordingly, the present case is among the first to demonstrate how dual VEGF-A/Ang-2 inhibition may be pragmatically integrated into surgical planning for Eales’ disease, with potential implications for future management strategies.

Case Report

A previously healthy 32-year-old White man presented with floaters and reduced visual clarity in the left eye. At presentation, he reported difficulty performing near tasks and reading due to floaters and blurred vision in the left eye, which interfered with daily activities. He had no history of systemic illness, infectious exposure, or autoimmune disease. He did not smoke, consume alcohol, or use recreational drugs. Best-corrected visual acuity (BCVA) at presentation was hand motion (≈2.3 logarithm of the minimum angle of resolution [logMAR]) in the left eye and 5/6 Snellen (≈0.08 logMAR) in the right eye.

Ultrasonography of the left eye demonstrated vitreous hemorrhage, vitreoretinal traction, and focal retinal detachment in the temporal quadrants. The right eye exhibited peripheral retinal changes and laser scars consistent with prior panretinal photocoagulation (PRP) in another hospital, possibly due to leakage and peripheral retinal changes. Fluorescein angiography confirmed peripheral ischemia and vascular leakage in the left eye.

Systemic evaluation – including QuantiFERON-TB Gold (interferon-gamma release assay for tuberculosis), Venereal Disease Research Laboratory testing, Borrelia antibody assessment, toxoplasmosis serology, and chest radiography – excluded tuberculosis, syphilis, Lyme disease, and toxoplasmosis. Other differential diagnoses, such as diabetic retinopathy, retinal vein occlusion, and alternative inflammatory vasculopathies, were considered and excluded on the basis of medical history, laboratory findings, and characteristic angiographic features. Accordingly, a diagnosis of bilateral Eales’ disease was established.

Initial short-term supportive therapy with troxerutin and etamsylate was administered to stabilize capillary permeability and reduce recurrent hemorrhage risk. These agents were discontinued after the initiation of definitive surgical management and systemic immunosuppressive therapy. Combined left-eye vitrectomy with phacoemulsification and posterior chamber intraocular lens (IOL) implantation was performed, along with endolaser photocoagulation and 15% sulfur hexafluoride (SF6) gas tamponade.

Subsequent bilateral fluorescein angiography confirmed areas of ischemia and mild vascular leakage in both eyes. PRP was completed in the right eye in 2 sessions. Systemic immunosuppression was initiated with azathioprine 50 mg twice daily (Imuran®, Aspen Pharma, Dublin, Ireland); however, treatment was discontinued after 2 months because of arthralgia. Therapy was subsequently switched to methotrexate 7.5 mg once weekly (Methotrexate®, Koçak Farma, Istanbul, Türkiye), with folic acid 5 mg once weekly (Acidum folicum®, Teva, Petah Tikva, Israel) administered on the day after methotrexate. Routine safety monitoring, including complete blood count and liver function tests (alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, and bilirubin), was performed every 4 to 6 weeks. All parameters remained within normal limits throughout follow-up.

During disease reactivation in the right eye, intravenous methylprednisolone 500 mg/day was administered for 3 consecutive days (Prednol-L®, Mustafa Nevzat, Istanbul, Türkiye), followed by an oral taper with methylprednisolone tablets (Medrol®, Pfizer, New York, NY, USA) initiated at 32 mg/day with gradual dose reduction. Concomitant proton pump inhibitor prophylaxis was prescribed to reduce gastrointestinal risk (pantoprazole 40 mg/day, Controloc®, Takeda, Zurich, Switzerland). Additionally, topical dexamethasone 1% eye drops (Maxidex®, Novartis, Basel, Switzerland) were initiated; faricimab 6 mg was administered for stabilization, 8 days prior to surgical management. A second vitrectomy with phacoemulsification, posterior chamber IOL implantation, fibrovascular tissue removal, 360° endolaser photocoagulation, and SF6 tamponade was performed in the right eye.

At final follow-up – 18 months after left-eye vitrectomy and 12 months after right-eye vitrectomy – BCVA had improved to 5/5 Snellen (0.00 logMAR) in both eyes. Baseline optical coherence tomography (OCT) images and retinal thickness maps obtained at presentation are shown in Figure 1. Post-treatment OCT scans and retinal thickness maps at final follow-up are presented in Figure 2. Table 1 summarizes the chronological sequence of ophthalmic interventions, systemic therapies, and corresponding visual outcomes (BCVA, logMAR) during the 18-month follow-up course for this 32-year-old man with bilateral Eales’ disease.

Discussion

Eales’ disease is uncommon outside endemic regions and is frequently misdiagnosed or detected at advanced stages [4,9,17]. The present case demonstrates bilateral involvement with asymmetrical progression and emphasizes the importance of phase-specific management.

Current evidence supports surgical intervention during the proliferative phase. Murillo López et al [9] reported that approximately one-third of patients with Eales’ disease require vitrectomy due to vitreous hemorrhage or retinal detachment. Ersöz et al [10] observed improved visual acuity in more than 70% of cases and anatomical success in over 90% of eyes after pars plana vitrectomy. In the present case, early surgical intervention in the left eye effectively halted disease progression and preserved full visual acuity.

The role of anti-VEGF therapy in Eales’ disease remains controversial. Mehboob et al [11] reported regression of neovascularization after bevacizumab administration, whereas Patwardhan et al [12] observed persistent surgical requirements and a potential increase in tractional retinal detachment. Bevacizumab and ranibizumab, both VEGF-A–specific inhibitors, are the most frequently reported agents; however, treatment responses have been inconsistent, and an elevated risk of tractional progression has been described. These considerations informed the selection of faricimab, which provides broader vascular stabilization via dual inhibition of VEGF-A and Ang-2 [11,13–15]. Ang-2 destabilizes endothelial junctions, increases vascular permeability, and acts synergistically with VEGF to promote pathological neovascularization [16]. Simultaneous blockade of VEGF-A and Ang-2 allows faricimab to exert a more comprehensive vascular-stabilizing effect. This mechanism is particularly relevant in inflammatory retinal vasculopathies such as Eales’ disease, in which vascular leakage and fibrovascular proliferation coexist [13,15].

Dual-pathway inhibition likely contributed to the favorable intraoperative conditions [13,15] observed in the present case, including reduced intraoperative bleeding, facilitated fibrovascular tissue dissection, and improved retinal stabilization after a single preoperative injection. Importantly, our case is among the first to demonstrate faricimab feasibility in a rare retinal vasculitis. In contrast to bevacizumab, which has been associated with variable outcomes and an increased risk of tractional progression, faricimab may represent a promising adjunctive therapy when surgery is planned in advanced bilateral disease [13–16].

In addition to anatomic and visual outcomes, the patient’s psychological well-being improved after bilateral disease stabilization. Confidence in visual prognosis was restored, normal daily activities were resumed, and functional status remained stable. Although these observations are anecdotal, validated instruments such as the National Eye Institute Visual Function Questionnaire–25 have demonstrated that vision-related quality of life improves after successful surgical stabilization of chronic retinal disease [18].

This report is limited by its single-patient design, which restricts the generalizability of the findings. Although excellent bilateral outcomes were achieved with pars plana vitrectomy, systemic immunosuppression, and adjunctive faricimab, these results cannot be directly extrapolated to the broader Eales’ disease population. Larger case series and controlled studies are required to determine whether dual VEGF-A/Ang-2 inhibition provides consistent benefit during surgical planning and long-term management of this rare vasculitis. Future reports incorporating standardized patient-reported outcome measures may further strengthen the evidence base.

Conclusions

This case demonstrates that early pars plana vitrectomy, combined with systemic immunosuppression, can result in excellent bilateral visual recovery in Eales’ disease. A distinguishing feature of this report is the successful adjunctive use of faricimab, a dual inhibitor of VEGF-A and Ang-2, which provided favorable intraoperative conditions and vascular stabilization before surgery. These findings emphasize the importance of phase-specific management, including timely intervention during the proliferative stage, sustained systemic immunomodulation, and selective anti-VEGF therapy guided by vascular biology. Although interpretation is limited by the single-case design, the results highlight a novel therapeutic approach and support further prospective evaluation of dual-pathway anti-VEGF therapy in retinal vasculitis.