Eales Disease

Overview

Eales disease is an idiopathic occlusive peripheral periphlebitis (inflammation and obliteration of peripheral retinal veins). It is rare in White populations but is a significant cause of visual morbidity among young South Asian males (typically 20–40 years of age). It is bilateral in most cases, though often asymmetrical, and is considered a diagnosis of exclusion after other causes of retinal vasculitis are ruled out.

— Kanski's Clinical Ophthalmology, 10th ed.

Aetiology

Idiopathic — the exact cause remains unknown.
Tubercular protein hypersensitivity is the most widely proposed mechanism: Mycobacterium tuberculosis antigens are thought to trigger a delayed-type hypersensitivity reaction in the peripheral retinal vessels, even in the absence of active systemic TB. PCR for TB DNA has been detected in vitreous specimens of some patients.
Oxidative stress and immunological dysregulation may play additional roles.
Evidence for TB association is conflicting; some patients are classified as having true tubercular vasculitis, while others have truly idiopathic disease.

Pathology & Stages

The disease passes through three overlapping stages:

Stage	Features
1 — Inflammatory	Perivenous sheathing (periphlebitis), superficial retinal haemorrhages, cotton-wool spots, mild anterior uveitis
2 — Occlusive	Branch retinal vein occlusion; peripheral capillary non-perfusion; microaneurysms, vascular tortuosity, shunts
3 — Neovascular	Retinal neovascularization (NVE/NVD) at the junction of perfused and non-perfused retina; recurrent vitreous haemorrhage (in ~1/3 of eyes)

Clinical Features

Symptoms

Floaters or sudden, painless visual loss due to vitreous haemorrhage
Neurological features (myelopathy, hemiplegia) have been reported rarely — Eales disease appears under spinal cord disorders in some neurology texts

Fundus Findings

Peripheral periphlebitis: vascular sheathing, perivascular exudates
Peripheral capillary non-perfusion: leads to neovascularization at the ischaemic border
Vitreous haemorrhage: often recurrent and usually self-limited, absorbing over weeks
Macular involvement: uncommon but possible

Complications

Tractional retinal detachment
Macular epiretinal membrane
Neovascular glaucoma
Cataract

Investigations

Fluorescein angiography (FA): key investigation — identifies periphlebitis, areas of capillary non-perfusion, and neovascularization; wide-field imaging is especially valuable
OCT: assess macular pathology
Investigations to exclude other causes: chest X-ray, Mantoux/PPD, QuantiFERON-TB Gold, ACE level (sarcoidosis), FBC with haemoglobin electrophoresis (haemoglobinopathies), VDRL/TPHA (syphilis), ANA/ANCA

Fundus Images

Clinical appearance (Kanski's, Fig. 13.59):

Eales disease — peripheral vascular sheathing and occlusion

Vascular occlusion, haemorrhage and laser scars (C)

FA demonstrating areas of capillary non-perfusion and neovascularization (D)

From the literature:

Wide-field FFA: peripheral periphlebitis, 360° capillary non-perfusion, neovascularization with leakage

Color fundus (left): silver-wire sclerosed peripheral vessels + NVE. FFA (right): peripheral ischaemia and hyperfluorescent leakage

Treatment

Inflammatory Stage

Topical, periocular, systemic, or intravitreal corticosteroids to suppress active vasculitis
Antitubercular therapy (ATT): controversial but advocated by many (especially in South Asian populations); recommended when QuantiFERON/Mantoux is strongly positive, or active TB is suspected; ATT with steroids reduces recurrence risk

Occlusive/Neovascular Stage

Scatter (sectoral) photocoagulation or cryotherapy to non-perfused peripheral retina — reduces neovascular stimulus (analogous to treatment of diabetic retinopathy)
Intravitreal anti-VEGF (bevacizumab, ranibizumab): widely used for active neovascularization

Surgical Stage

Pars plana vitrectomy: for persistent non-clearing vitreous haemorrhage, tractional retinal detachment, or macular epiretinal membrane

Prognosis

Generally good for central vision if macular involvement is absent and treatment is prompt
Recurrent vitreous haemorrhages are the main cause of morbidity; most clear spontaneously
Late complications (tractional detachment, neovascular glaucoma) carry a worse prognosis

Key Differentials

Condition	Distinguishing Feature
Sarcoidosis	Hilar lymphadenopathy, elevated ACE, "candle wax drippings"
Tuberculosis (ocular)	Active systemic TB, choroidal granulomas
Sickle cell retinopathy	Haemoglobin electrophoresis abnormal, "sea-fan" NVE
Pars planitis	Snowball/snowbank vitreous opacities, younger patients
Behçet disease	Oral/genital ulcers, arteriolar as well as venous involvement

— Kanski's Clinical Ophthalmology 10th ed.; Wills Eye Manual

Recent evidence note: Two 2026 systematic reviews from the International Uveitis Study Group (IUSG ReViSe study) address work-up and treatment in retinal vasculitis broadly, including Eales disease [PMID 40972796, PMID 40983164]. These may update protocols for investigation and immunosuppressive management.

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