gms | German Medical Science

Eales disease is characterized by distinct alterations of the blood vessels in the retinal periphery. It occurs predominantly in otherwise healthy men between 20 and 40 years of age and affects both eyes in most cases. On the Indian subcontinent, Eales disease is one of the most frequent retinal disorders; in contrast, it is rarely seen in Europe or the USA. The aetiology of the disease is still unknown. It has been associated with tuberculosis, hypersensitivity to tuberculoprotein, autoimmunity to retinal autoantigens, certain HLA-antigens, oxidative stress of the retina and neurological disorders in different studies; a multifactorial aetiology seems most likely.

The hallmark of Eales disease is active vasculitis or "burned-out" periphlebitis (sheating) of the peripheral retinal veins. The disease can proceed to retinal ischemia, permanent alterations of the vascular bed in the retinal periphery, development of optic disc or peripheral neovascularisations, vitreous haemorrhage, epiretinal gliosis, secondary macular changes and tractional retinal detachment. The diagnosis of Eales disease is one of exclusion. Other causes of vasculitis, vaso-occlusive disorders of the retinal periphery and vitreous haemorrhage - e.g. sarcoidosis, pars planitis, Behcet`s disease, syphilis, sickle-cell retinopathy, diabetic retinopathy, venous occlusive disease - have to be excluded. In Eales disease, the central retinal vessels and the choroidal vasculature are usually not affected and there is only mild anterior segment inflammation. Fluorescein angiography can aid the diagnosis by demonstrating ischemia of the retinal periphery and typical "sea fan" neovascularisations bordering the ischemic retinal areas.

Treatment of Eales disease is adjusted to the disease stage at clinical presentation. In active periphlebitis, systemic or local anti-inflammatory agents, e.g. steroids, are usually recommended. Peripheral retinal ischemia and neovascularisations are treated with photocoagulation of the ischemic retina, panretinal photocoagulation or direct treatment of the neovascularisations. Secondary complications of a possible proliferative vitreoretinopathy, e.g. persistent vitreous haemorrhage, epiretinal membranes or tractional retinal detachment, are treated with pars plana vitrectomy. The prognosis depends on the stage of the disease at presentation: reading ability and a useful visual field can usually be maintained as the disease process is generally controlled with adequate therapy, and the central retina is only affected in the late stage of the disease.