Abstract
The ophthalmologist has direct visual access to inflamed vessels when examining the retina, and "vasculitis" in ophthalmology has so far mainly referred to retinal vasculitis. In the past few years the means to explore vasculitis in the ocular sphere have improved. Indocyanine green angiography now enables the analysis of choroidal inflammatory vasculopathy as well as vasculitis of the sclera (scleritis) and episclera (episcleritis) in addition to retinal vasculitis. Because vasculitis detected by the ophthalmologist can be the presenting sign of a systemic disease and has to be approached in a multidisciplinary fashion, the emerging term "ocular vasculitis," instead of retinal vasculitis, should be used in the future. The term covers vasculitis affecting all structures of the eye and the periocular tissues as detailed in this article. The ocular findings have to be integrated within the established and accepted classification of systemic vasculitis, which is divided into primary vasculitides, where the vessel itself is the target of the inflammatory reaction, and secondary vasculitides, caused by other inflammatory processes. This review will deal with recently published articles on ocular vasculitis, including its clinical aspects, its link with systemic diseases, and its investigation and management. The discussion will be conducted within the framework of the new classification put forward here.
Novel imaging techniques such as indocyanine green angiography have made it possible to explore inflammation of choroidal vessels and of scleral vasculitis in addition to retinal vasculitis, contributing to the global concept of ocular vasculitis. It has been shown, in particular, that the choriocapillaris, a vascular structure adjacent to the retina, can be the site of a primary inflammatory vasculopathy unrecognized so far. Most of the recent articles cited, however, deal not so much with new findings but with the integration of ocular pathologic changes into the systemic diseases they are part of. New knowledge about disease mechanisms and novel therapeutic modalities with biologic agents cited in this review are coming from other fields but have contributed to progress in the management of ocular vasculitis.
New investigational techniques of vasculitis in ocular structures other than the retina have contributed to the development of the global concept of ocular vasculitis. This review shows the importance of promoting a comprehensive and global classification of ocular vasculitis compatible with the concepts accepted for systemic vasculitis to contribute to its multidisciplinary approach.