Eales disease is an idiopathic peripheral retinal vasculitis, typically affecting young, otherwise healthy adult men, characterized by peripheral retinal vascular inflammation and occlusion that predisposes to recurrent vitreous hemorrhage.
It remains a diagnosis of exclusion, made only after a careful search for the many recognized causes of retinal vasculitis and peripheral vascular occlusion has failed to identify a specific underlying etiology, despite decades of study into a possible infectious or immunologic trigger.
Because it is defined partly by what it is not, a systematic and thorough exclusion of mimicking conditions is inseparable from actually making the diagnosis correctly.
The disease shows a notably higher reported incidence in certain regions, particularly parts of South Asia, than elsewhere, a geographic pattern that has fueled much of the long-standing interest in an infectious or environmental trigger.
Clinical Course
The disease classically progresses through overlapping stages: an initial phase of peripheral retinal vasculitis, most often periphlebitis, followed by peripheral vascular non-perfusion, and eventually neovascularization arising from the areas of ischemia.
Once neovascular vessels form, they are fragile and prone to bleeding into the vitreous, which is what produces the recurrent vitreous hemorrhage that is often the presenting complaint bringing a young, previously asymptomatic patient to medical attention.
The disease is frequently bilateral, though the two eyes can be affected asymmetrically and at different points in their individual disease course, meaning one eye may be actively hemorrhaging while the other shows only early, asymptomatic peripheral vasculitis.
Clinical Presentation
- Sudden painless vision loss or floaters from vitreous hemorrhage, often the presenting complaint in a young, healthy adult male with no prior ocular history
- Peripheral retinal vascular sheathing and inflammation on exam of the fellow or less severely affected eye, when the view allows adequate visualization
- Peripheral neovascularization, best appreciated on fluorescein angiography, arising at the border of perfused and non-perfused peripheral retina
- A demographic pattern strongly skewed toward young adult men, typically in their twenties or thirties, a distinctive epidemiologic clue that helps distinguish Eales disease from many of its mimics
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From Choroida — the team behind this siteDifferential Diagnosis
Diabetic retinopathy can produce a broadly similar picture of peripheral non-perfusion and neovascularization, but typically occurs in an older patient with an established diabetes history and a different, more central pattern of vascular change.
Retinal vein occlusion, sickle cell retinopathy, and sarcoidosis-associated retinal vasculitis, discussed in relation to ocular sarcoidosis in its own dedicated article on this site, all need to be actively excluded given their overlapping potential to produce peripheral vasculitis and non-perfusion.
Infectious causes, particularly tuberculosis, have long been proposed as a possible trigger for Eales disease specifically, and a history and workup for latent or active tuberculosis exposure is a standard part of the evaluation in endemic regions, even though the exact causal relationship remains unproven.
Diagnostic Evaluation
Fluorescein angiography is essential for mapping the extent of peripheral non-perfusion and identifying areas of active or subclinical neovascularization, guiding both diagnosis and subsequent laser treatment planning.
A thorough systemic workup, including screening for diabetes, sickle cell disease, sarcoidosis, tuberculosis, and other systemic vasculitides, is necessary to support the diagnosis of exclusion, since Eales disease cannot be confidently diagnosed until these other causes have been reasonably ruled out.
B-scan ultrasonography, discussed in its own dedicated article on this site, is useful when vitreous hemorrhage precludes an adequate direct view of the retina, helping assess for an underlying retinal detachment that would change the urgency and approach to treatment.
Management
Peripheral scatter laser photocoagulation is applied to areas of retinal non-perfusion and neovascularization, following the same general treatment logic used for other ischemic retinal diseases, aiming to reduce the angiogenic drive behind ongoing neovascularization and hemorrhage risk.
Corticosteroids or other immunosuppressive therapy may be used during the active vasculitic phase to control inflammation, particularly when active periphlebitis is present alongside the ischemic and neovascular findings.
Vitrectomy is reserved for eyes with non-clearing vitreous hemorrhage, tractional retinal detachment, or other complications that do not resolve with laser and medical management alone, following similar surgical principles to other proliferative retinal vascular diseases.
Because both eyes are frequently involved even when only one is currently symptomatic, careful examination and monitoring of the fellow eye is a standard, essential part of managing every confirmed case.
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From Choroida — the team behind this siteReferences
- Biswas J, Sharma T, Gopal L, et al. Eales disease: an update. Survey of Ophthalmology.
- American Academy of Ophthalmology. Basic and Clinical Science Course, Section 12: Retina and Vitreous.
- Saxena S, Kumar D. Eales disease: an update. Journal of Postgraduate Medicine.
