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Eales Disease: Causes, Symptoms and Treatment

Eales disease is a rare inflammatory condition affecting the peripheral retinal blood vessels, primarily in young men, and can lead to vision loss.

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Things worth knowing about "Eales Disease"

Eales disease is a rare inflammatory condition affecting the peripheral retinal blood vessels, primarily in young men, and can lead to vision loss.

What is Eales Disease?

Eales disease is a rare, idiopathic inflammatory and obliterative condition of the peripheral retinal blood vessels. It is characterized by vasculitis of the retinal veins, leading to recurrent vitreous hemorrhages, retinal ischemia, and neovascularization. In severe cases, it can result in permanent vision loss. The condition was first described in 1880 by British ophthalmologist Henry Eales and predominantly affects young men between the ages of 20 and 40.

Causes

The exact cause of Eales disease remains incompletely understood. Several contributing factors have been proposed:

Tuberculosis association: A strong link with Mycobacterium tuberculosis has been identified, particularly in regions where tuberculosis is endemic. A hypersensitivity reaction to mycobacterial antigens is considered a possible trigger.
Immune-mediated mechanisms: An aberrant cell-mediated immune response targeting retinal vessel walls may drive the inflammatory process.
Oxidative stress: Elevated levels of free radicals and impaired antioxidant defenses have been documented in affected individuals.
Genetic predisposition: Certain HLA (Human Leukocyte Antigen) subtypes may increase susceptibility to the disease.

Symptoms

Eales disease typically progresses through three stages, each with distinct clinical features:

Stage 1 - Periphlebitis: Inflammation of peripheral retinal veins, often asymptomatic or associated with mild visual disturbance.
Stage 2 - Occlusion: Progressive occlusion of retinal vessels leads to ischemia (reduced oxygen supply) of the peripheral retina.
Stage 3 - Proliferation: Formation of fragile new blood vessels (neovascularization) that are prone to bleeding. Patients may experience sudden blurred vision, floaters (small dark spots or threads in the visual field), or flashes of light (photopsia).
In advanced cases: retinal detachment and irreversible vision loss may occur.

Diagnosis

Diagnosis is primarily based on ophthalmological evaluation:

Fundoscopy (fundus examination): Direct visualization of retinal hemorrhages, vascular changes, and neovascularization.
Fluorescein angiography: Imaging of retinal vessels using a fluorescent dye to detect leakage and vascular occlusions.
Optical Coherence Tomography (OCT): High-resolution cross-sectional imaging of the retinal layers.
Laboratory tests: Screening for tuberculosis (tuberculin skin test or IGRA), complete blood count, and inflammatory markers to exclude other causes.
Differential diagnosis: Other conditions such as diabetic retinopathy, sarcoidosis, and sickle cell retinopathy must be ruled out.

Treatment

There is currently no curative treatment for Eales disease. Management is tailored to the stage and severity of the condition:

Medical Treatment

Corticosteroids: Systemic or periocular corticosteroids are used to reduce inflammation during the active phase.
Antitubercular therapy: In cases with confirmed or suspected tuberculosis association, a combination antibiotic regimen targeting Mycobacterium tuberculosis may be initiated.
Antioxidant supplementation: Vitamins C and E and other antioxidants have been explored as adjunctive therapy to reduce oxidative stress.

Interventional Treatment

Laser photocoagulation: Ablation of ischemic retinal areas to prevent further neovascularization and reduce the risk of vitreous hemorrhage.
Vitrectomy: Surgical removal of blood-filled vitreous in cases of severe or non-clearing hemorrhage.
Anti-VEGF injections: Intravitreal injections of anti-vascular endothelial growth factor agents (e.g., bevacizumab) can help suppress abnormal vessel growth.

Prognosis

The prognosis of Eales disease varies widely. Many patients respond well to early treatment and maintain functional vision. However, delayed diagnosis or severe disease course can lead to permanent visual impairment. Regular ophthalmological follow-up is essential to monitor disease activity and prevent complications.

References

Das, T. et al. - Eales Disease: Current Concepts in Pathophysiology, Diagnosis and Management. Indian Journal of Ophthalmology, 2021.
Saxena, S. et al. - Eales Disease. Acta Ophthalmologica Scandinavica, 2000; 78(3): 249-254.
Agarwal, A. - Gass' Atlas of Macular Diseases, 5th Edition. Elsevier, 2012.

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