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Retinal Vasculitis: a Framework and Proposal for a Classification System

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Retinal Vasculitis: a Framework and Proposal for a Classification System survey of ophthalmology 66 (2021) 54e67 Available online at www.sciencedirect.com ScienceDirect journal homepage: www.elsevier.com/locate/survophthal Major review Retinal vasculitis: A framework and proposal for a classification system Ghazala A. Datoo O’Keefe, MDa,*, Narsing Rao, MDb a Assistant Professor of Ophthalmology, Section of Vitreoretinal Surgery and Diseases, Section of Uveitis and Vasculitis, Emory University School of Medicine, Atlanta, Georgia, USA b Grace and Emery Beardsley Professor and Chair, USC Department of Ophthalmology; USC Roski Eye Institute; Director of the Uveitis Service and Ophthalmic Pathology Laboratory, The Keck School of Medicine, University of Southern Califonia, Los Angeles, California, USA article info abstract Article history: Retinal vasculitis, a poorly understood process involving inflammation or ischemia of the Received 24 July 2019 retinal vessel wall, may occur in association with a systemic process, although it can also Received in revised form 12 May be isolated to the retina. Because of the limited ability to perform histopathological studies 2020 on retinal vessels, there is no gold standard for diagnosis. Thus, there is utility in creating a Available online 22 May 2020 classification system for retinal vasculitis and improving diagnostic strategies for this disease. We provide a framework for understanding retinal vasculitis based on size, Keywords: location, and etiology. We hope that this information can be implemented in the clinical retinal vasculitis setting to provide some diagnostic strategies for this often confusing entity. vasculitis ª 2020 Elsevier Inc. All rights reserved. uveitis inflammation birdshot infectious uveitis noninfectious uveitis systemic vasculitis 1. Introduction associated with systemic vasculitis as there is not easy access to tissue specimens. While retinal vasculitis may occur in Retinal vasculitis, or inflammation of the blood vessel wall, is association with systemic vasculitis, it can also be isolated to a poorly understood entity. This is in part due to the lack of the retina. It may also occur secondary to infectious or histopathological evidence, a gold standard for diagnosing noninfectious intraocular inflammation. Usually when it is any form of vasculitis, consisting of inflammatory cell infil- associated with an infectious etiology, there are associated tration of the vessel wall. It is a clinical diagnosis associated changes to the retina including, but not limited to, retinal with inflammation or ischemia of the retina; however, histo- whitening or a chorioretinitis.56 Fluorescein angiography is pathological analysis cannot be fulfilled unless it is also used to evaluate the presence and extent of retinal vasculitis, Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. * Corresponding author: Ghazala A. Datoo O’Keefe, MD, Emory Eye Center, 1365 Clifton Road B, Suite B2400C, Atlanta, GA, 30322. E-mail address: [email protected] (G.A. Datoo O’Keefe). 0039-6257/$ e see front matter ª 2020 Elsevier Inc. All rights reserved. https://doi.org/10.1016/j.survophthal.2020.05.004 survey of ophthalmology 66 (2021) 54e67 55 but cannot reliably differentiate between vascular damage manifestations associated with giant cell arteritis and from other causes such as diabetic retinopathy or vascular Takayasu arteritis in the literature correspond to sequelae of occlusions, and true vasculitis.96 Ultrawidefield (UWF) imag- poor perfusion of the retina and other ocular structures from ing demonstrates areas of varying peripheral retinal leakage, large-vessel disease affecting the internal carotid artery or the the implication of which, to the management of disease, is ophthalmic artery branching from it. Examples of these currently uncertain. In 2005, the working group for the Stan- include ocular ischemic syndrome, iris neovascularization, dardization of Uveitis Nomenclature reported that “there was anterior ischemic optic neuropathy, neovascular glaucoma, consensus that the definition of retinal vasculitis required and hypertensive retinopathy.92 They do not manifest as more work”.59 Despite its relatively frequent occurrence in retinal vasculitis.The ocular ischemic syndrome from large- uveitis and related intraocular inflammations, our under- vessel disease84 can present with clinical findings of non- standing of the diagnosis and treatment of this entity is poor. perfusion and peripheral retinal ischemia and not as a retinal Vasculitis, as a diagnosis, was first described by Kussmaul vasculitis. in 1866 in the setting of a patient who died of a disease marked Posterior and anterior ciliary vessels represent medium by fever and progressive weakness, which was later called size vessels. Sequelae of medium-vessel vasculitis manifest polyarteritis nodosa.61 Despite being recognized for 150 years, primarily as a scleritis and peripheral ulcerating keratitis, our understanding of the mechanism behind the inflamma- as well as uveitis associated with polyarteritis nodosa and tion is incomplete. In an attempt to understand various sys- Kawasaki disease.29 The medium-vessel vasculitides are temic inflammatory vascular diseases, a nomenclature was more likely to be acute, necrotizing in nature61 and have a proposed for systemic vasculitis by Zeek in 1952123 by classi- high mortality rate.41,44 Similar to large-vessel disease, fying the necrotizing angiitides which was then elaborated medium-vessel disease does not appear to cause retinal upon by Fauci in 1978 using information regarding histopa- vasculitis; however, it can cause infarction of the choroid or thology and etiology of disease.41 Vessel size was first used in retina from either perfusion abnormalities or inflammation addition to this existing classification in 1994 at the Chapel involving the choroidal vasculature of Haller and Sattler Hill Consensus Conference60 as well as in a subsequent revi- layers.38,55 sion.61 More recently, an attempt has been made to create a Retinal vasculitis then is mostly a disease of small vessels, more clinically relevant integrated nomenclature for the sys- seen clinically as fluffy white sheathing along the vessels and temic vasculitides.95 usually associated with vitritis.50 It is suggested that circu- There has been a lack of similar framework in the lating immune complexes are involved in the pathogenesis of ophthalmology literature, likely due to the segregation of the this disease, but data are limited.11,120 It has also been induced disease in the eye and the limited ability to perform histo- experimentally by provoking an immune response against pathological studies on retinal vessels. The diagnosis is made rhodopsin kinase, or S antigen.99 Although, as stated previ- based on a clinical examination of the retinal vasculature, ously, vessel leakage on fluorescein angiography cannot reli- which may demonstrate sheathing of the retinal vessels, focal ably distinguish between vasculitis and vasculopathies; fluffy white cuffs around the vasculature, nodular periarter- clinically, the diagnosis of retinal vasculitis is supported by iolar plaques, and the presence of cells in the vitreous cavity.50 fluorescein angiography that shows vascular leakage with or In the absence of histopathological confirmation and based on without macular edema.120 Retinal vasculitis can be confusing clinical features and retinal vascular imaging, we believe there because the complications seen can be mimicked by nonin- is utility in thinking about retinal vasculitis based on the flammatory disorders; for example, noninflammatory vessel size and type and agree in principal with the 2016 in- vascular sclerosis can be confused with inflammatory tegrated nomenclature by Prete and colleagues.95 We propose sheathing.56 There are no clear-cut features that distinguish a framework for understanding retinal vasculitis based on the vasculitis from vasculopathy, although certain findings may framework set forth by prior nomenclatures for systemic assist in the differentiation. The presence of vitreous cells vasculitides, realizing that retinal vasculitis frequently occurs may point toward a vasculitis. Concurrent diagnoses of dia- in the absence of systemic vasculitis.98 We believe that this betes mellitus and hypertension in a patient with sudden framework will be useful for the understanding of retinal onset of vision loss and a clinical findings of four quadrants of vasculitis, whether idiopathic or in the setting of systemic dot-blot hemorrhages is more likely to be a retinal vein oc- disease. In addition, we will recommend some diagnostic clusion. UWF imaging is being used increasingly frequently to strategies for this often confusing entity. evaluate peripheral retinal findings in patients. Leakage of peripheral retinal vessels has been documented in up to 42% of patients with anterior uveitis, 44% of whom did not have 2. Background clinically active disease.28 Cystoid macular edema associated with anterior uveitis was more common in those with pe- Before the creation of a framework, it is vital to understand ripheral retinal leakage, and 44% of those with peripheral that we encounter mostly small-vessel vasculitis in the retina retinal leakage had a specific etiology of their anterior uveitis as a result of the histological makeup of the retinal vascula- including ankylosing spondylitis, juvenile idiopathic arthritis, ture. Large-vessel vasculitis such as aortitis, giant cell arter- and tubulointerstitial nephritis and uveitis.28 More
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