Ординатура / Офтальмология / Учебные материалы / Uveitis Text and Imaging Text and Imaging Text and Imaging 2009

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INTRODUCTION

The term of vasculitis, in the field of ophthalmology, is used in different senses including the proper internal medicine sense of a histologically proven vasculitis (primary vasculitis due to inflammation of the vessel wall) (Figures 1A-C), as well as, most often, in the sense of a clinical sign among other signs seen and evidenced by ophthalmological investigational techniques such as fluorescein angiography or simply fundoscopy. (Figures 2A and B) In the latter situation the term of vasculitis very often refers to secondary vessel changes without histologic evidence of proper vessel wall inflammation and should then be called inflammatory vasculopathy or similar. Both conditions can however be included in an all encompassing classification by indicating when vasculitis is used in its original proper sense. In this chapter this will be specified by an asterisk behind the term vasculitis. Whenever this is not specified, vasculitis is used in the sense of inflammatory vasculopathy.

THE CONCEPT OF OCULAR VASCULITIS VERSUS RETINAL VASCULITIS

The ophthalmologist has direct visual access to inflamed vessels when examining the retina and therefore the term of vasculitis in ophthalmology refers classically to retinal vasculitis and most of the published work on vasculitis in the ocular field concerns

retinal vasculitis. Classically retinal vasculitis was divided into entities localised to the retina on one hand and into systemic diseases involving the eye on the other hand. Vasculitis in the ocular sphere is however not limited to the retina but can also touch the choroid, the sclera, the periocular tissue and the ocular adnexae. A patient presenting with scleritis due to Wegener’s granulomatosis (a vasculitis) (Figure 3) should fit into the same global classification as a case of Behçet’s uveitis (Figure 4) or a case of Wegener’s retinal vasculitis (Figure 1). Therefore the more global concept of ocular vasculitis including retinal vasculitis is useful and should be considered as an emerging terminology because it is clinically more relevant for the appraisal of vasculitis in a multidisciplinary approach.

Such a terminology is relevant as we now have the means to explore efficiently not only retinal vasculitis as was the case so far with fluorescein angiography but other vasculitic ocular involvement. Thanks to indocyanine green angiography (ICGA) we are now also able to explore choroidal inflammatory vasculopathy or vasculitis* that was inaccessible before and1 ICGA makes it also possible to analyse more precisely the vasculitic process in anterior scleritis.2

At the level of the choroid, this new technology using ICG has fundamentally changed our appraisal of intraocular inflammation and vasculitis as has allowed us to have imaging access to the inflamed choroidal vessels. The technique allowed to differen-

Figures 1A-C: Example of retinal vasculitis* in a case of Wegener’s granulomatosis: Extensive retinal vasculitis* in the posterior pole (A), that was occlusive in the periphery needing laser photocoagulation (B) in a patient later diagnosed as Wegener’s granulomatosis following the resection of a lung inflammatory pseudo-tumour (C) associated with very high levels of c-ANCA. Wegener’s granulomatosis is a primary vasculitis* where the inflammation directly involves the vessel wall and is classified in the small and medium-sized vasculitis

tiate between at least two choroidal inflammatory vasculopathies or vasculitis (*), the first involving the choriocapillaris circulation at the origin of a group of diseases classified within the new concept of primary inflammatory choriocapillaropathies3 and the second involving larger choroidal vessels defined as stromal choroidal inflammatory vasculopathies or vasculitis (*).4

Because the ophthalmologist can detect retinal or choroidal vasculitis or other lesions caused by a vasculitic process such as episcleritis, scleritis and orbital disease that may all be part of a systemic disease, it is important to classify and integrate ocular vasculitis (*) within the accepted classification of systemic vasculitis.

In 1992 the Chapel Hill consensus conference on the nomenclature of systemic vasculitis generated a uniformly accepted classification of primary systemic vasculitides based on histopathology showing that the vessel wall was the primary target. These entities were differentiated from secondary vasculitides in which vessel inflammation may be prominent but is a phenomenon secondary to other inflammatory processi. Primary systemic vasculitides were subdivided according to the size of the vessel principally involved into large, medium-sized and small vessel vasculitis.

The emerging term of ocular vasculitis should encompass episcleritis, scleritis including peripheral ulcerative keratitis (PUK), retinal vasculitis, choroidal vasculitis, optic nerve vasculitis or papillitis and should be extended to the orbit and adnexae as all these locations can produce ocular signs induced by vasculitis and diagnosed by the ophthalmologist.

Such a global approach of the vasculitic process in the ocular field is certainly going to be clinically more relevant in order to initiate a comprehensive multidisciplinary approach when this is necessary.

Ocular vasculitis following the accepted classification of systemic vasculitis should likewise be divided into lesions caused by primary systemic vasculitis and lesions caused by secondary systemic vasculitis. In addition to systemic vasculitis entities that involve the eye, there are entities where the vasculitic process is or seems to be limited to the eye. The mechanism is suspected to be primary in some of these entities and they can provisionally be classified into the section of primary inflammatory vasculitis limited to the eye such as the primary choriocapillaro-

Figures 3A and B: Scleritis due to Wegener’s granulomatosis, a vasculitis* causing necrosis. The lower part of the visible sclera shows active inflammation, whereas the upper part shows blue necrotic sclera. The active part of the vasculitis is well delineated by anterior segment indocyanine green angiography showing leaking vessels in the area of active inflammation, that should be impermeable to ICG

pathies individualised recently.3 However the primary nature of the vascular insult is not proven yet and we don’t dispose of any histology and therefore the term of vasculitis should still be used without the asterisk. In most of these “local entities” the inflammation of ocular vessels is however secondary to another inflammatory mechanism such as in birdshot chorioretinopathy where the primary immune mediated mechanism is a granulomatous inflammatory reaction. Infections represent the other factors causing secondary inflammatory vasculopathy that may be limited to the eye such as acute retinal necrosis caused by the ubiquitous herpes viruses but which is occurring only in the eye.

Following this principle, a detailed classification of ocular vasculitis taking into account four main subclasses can be put forward: (1) primary vasculitis

Figure 4: Retinal vasculitis in a case of Behçet’s uveitis. This is a systemic secondary vasculitis as the inflammatory mechanism is not directed against the vessel wall but vessel dysfunction is secondary to another inflammatory process. Nevertheless the two types of vascular dysfunctions are advantageously classified in a single global system

limited to the eye, a group recently individualised with no histopathologic information yet, (2) systemic primary vasculitis* involving the eye, (3) secondary vasculitis (in the sense that vasculitis is responding or taking part secondarily in the inflammatory process but is not the primary target of the inflammation) limited to the eye and (4) the vast group of systemic secondary vasculitic entities involving the eye, whether immune mediated, infectious, drug-induced or neoplastic (Table 1). The entities from these 4 subclasses were listed under the different ocular structures from front to back when they cause lesions in these structures (Table 1).5 Although this classification is as complete as possible it is by far not exhaustive. As far as the ocular involvement of primary systemic vasculitis* is concerned this chapter followed the classification exposed in the article by Jennette and Falk based on the Chapel Hill consensus conference on the nomenclature of systemic vasculitis6 and the ocular counterpart article that looked at the ocular manifestations of primary systemic vasculitis entities.7

PRIMARY VASCULITIS OR INFLAMMATORY VASCULOPATHY LIMITED TO THE EYE

The availability of indocyanine green angiography made it possible to have new insights into choroidal

inflammation. It allowed to better classify choroiditis and understand disease mechanisms. Diseases such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multiple evanescent white dot syndrome (MEWDS), multifocal choroiditis (MFC) and serpiginous choroiditis, thought to be diseases of the retinal pigment epithelium, were shown to be due to inflammation of the choriocapillaris and all these diseases could be grouped together under the term of inflammatory choriocapillaropathies.3 It is still unknown whether the mechanism is a primary inflammation of the choriocapillaris or whether choriocapillaris involvement is secondary to an inflammatory process. Furthermore no histopathology of these diseases is available. The reversibility of lesions in some of these conditions (MEWDS, APMPPE ) speaks against vessel wall pathology (Figures 5A and B). However, other conditions such as serpiginous choroiditis could be resulting of direct vessel wall pathology as there is choriocapillaris necrosis and scarring (Figure 6). ICGA further identified the inflammatory eye diseases that caused inflammation to the choroidal stromal vessels in addition to retinal vasculitis. The new classification of choroiditis and choroidal inflammatory vasculopathies based on ICGA has been exposed in a recently published.8-10

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) is a rare bilateral condition including retinal arteritis, aneurysmal dilatations of the retinal and optic nerve head arterioles, neuroretinitis and uveitis that is limited to the eye and could be a primary inflammation of the retinal vessel wall. However, the association of IRVAN with allergic fungal sinusitis recently reported seems to indicated that a systemic hypersensitivity mechanism could be at the origin of IRVAN which would imply that the vasculitis is possibly secondary11 (Figure 7).

PRIMARY SYSTEMIC VASCULITIDES INVOLVING THE EYE AND ADNEXAE

Recent publications in this field are mostly review articles on the involvement of the eye and adnexae in primary systemic vasculitides or case reports. A crucial article is the extensive review of the ocular lesions in primary systemic vasculitides that follows the Chapel Hill Consensus Conference classification.6

Figures 5A and B: Multiple evanescent white dot syndrome (MEWDS). Most probably caused by a vasculitis of the choriocapillaris which is possibly primary. The involvement is selectively situated at the choroidal level where ICGA shows non-perfusion of geographically involved sectors that appear hypofluorescent (A), whereas fluorescein angiography is practically normal indicating normal retina (B). The benign course speaks, however, against a process directed directly against the choriocapillaris vessel wall. No histology is available to settle the question (Courtesy Dr Alessandro Mantovani, Como, Italy)

The authors realised the importance of integrating ocular vasculitis into this well established classification. For each of the large, medium-sized and small vessel vasculitides ocular manifestations have been compiled.6 McDonald et al describe a case of Takayasu’s arteritis in a child presenting with a bilateral uveitis and CME,