Chapter 1.2 - Chapter

Summary

Among primary form of eye vasculitis, Eales’ disease – also known as retinal periphlebitis or retinal perivasculitis is a rare inflammatory venous occlusive disease. If first cases were reported in Europe or north America, Eales’ disease is now more common in developing countries, probably because of public health issues and an association with Mycobacterium tubercolosis infection has been suggested. The pathological hallmark of the disease is the involvement of peripheral retinal vessels with a first stage of inflammation (i.e. acute phlebitis) that eventually leads to ischaemia, haemorrhages into the vitreous body and then neovascular proliferation due to circulatory deficiency. Patients may complain of sudden blurring of vision in one or both eyes, decreased vision or floaters, while focal neurological deficits can rarely occur, probably because of vasculitic involvement of cerebral arteries leading to clinical and subclinical strokes. Eales’ disease is considered as a diagnosis of exclusion and confirmed only after ruling out all possible infections and systemic aetiologies. Brain MRI or CT angiography is crucial because it may show multiple and irregular stenosis involving both anterior and vertebrobasilar circulation. Fundus oculi examination and fluorescein angiogram may reveal retinal vascular leakage. Finally, the treatment of Eales’ disease depends on the stage of the disease, so in the acute inflammatory stage oral corticosteroids are the mainstay of therapy. As other autoimmune disorders, immunosuppressive therapy, as Cyclosporine, Methotrexate and Azathioprine, may be employed with the aim of preventing relapses