Retinal vasculitis associated with primary ocular disorders
- Michael Tolentino, MD
Michael Tolentino, MD
- Director of Clinical Research
- Center for Retina and Macular Disease
- Associate Professor, Ophthalmology
- University of Central Florida
- Reza Dana, MD, MPH, MSc
Reza Dana, MD, MPH, MSc
- Claes Dohlman Professor of Ophthalmology
- Harvard Medical School
- Section Editors
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
- Jonathan Trobe, MD
Jonathan Trobe, MD
- Section Editor — Ophthalmology
- Professor of Ophthalmology and Visual Sciences
- Professor of Neurology
- University of Michigan Kellogg Eye Center
Retinal vasculitis is characterized by inflammation of the vessels of the retina . Positive findings on fluorescein examination may be diagnostic, with fluorescein angiography demonstrating vascular leakage, perivascular staining, and capillary nonperfusion.
The detection and characterization of retinal vasculitis may help in the diagnosis and management of a variety of disorders associated with ocular inflammation. These include systemic autoimmune disorders, some infectious diseases, and certain ocular processes.
The clinical features of retinal vasculitis occurring in association with ocular disorders will be reviewed here. Retinal vasculitis due to systemic autoimmune disorders and infectious agents and a classification of the vasculitides are discussed separately. (See "Retinal vasculitis associated with systemic disorders and infections" and "Overview of and approach to the vasculitides in adults".)
The classic symptom of retinal vasculitis is a painless decrease in vision. Other symptoms may include a blind spot from ischemia-induced scotomas or floaters from vitritis. With macular involvement, patients may present with metamorphopsia (change in shape of an object) or abnormalities in color vision. Retinal vasculitis can also be asymptomatic.
Physical examination — Abnormalities in the retinal vessels may be observed upon physical examination. Funduscopic findings include visible accumulation of inflammatory cells along vessel walls (vascular sheathing) (picture 1). The peripheral vessels are often more involved than central arterioles and venules, and there are frequently skip areas. With certain disorders such as sarcoidosis, lesions resembling candlewax drippings or extensive perivascular inflammation may also be observed.
- Stanford MR, Graham EM. Systemic associations of retinal vasculitis. Int Ophthalmol Clin 1991; 31:23.
- Leder HA, Campbell JP, Sepah YJ, et al. Ultra-wide-field retinal imaging in the management of non-infectious retinal vasculitis. J Ophthalmic Inflamm Infect 2013; 3:30.
- Kasp E, Graham EM, Stanford MR, et al. A point prevalence study of 150 patients with idiopathic retinal vasculitis: 2. Clinical relevance of antiretinal autoimmunity and circulating immune complexes. Br J Ophthalmol 1989; 73:722.
- George RK, Walton RC, Whitcup SM, Nussenblatt RB. Primary retinal vasculitis. Systemic associations and diagnostic evaluation. Ophthalmology 1996; 103:384.
- Howe LJ, Stanford MR, Edelsten C, Graham EM. The efficacy of systemic corticosteroids in sight-threatening retinal vasculitis. Eye (Lond) 1994; 8 ( Pt 4):443.
- Nussenblatt RB, Palestine AG, Chan CC. Cyclosporin A therapy in the treatment of intraocular inflammatory disease resistant to systemic corticosteroids and cytotoxic agents. Am J Ophthalmol 1983; 96:275.
- Shah SS, Lowder CY, Schmitt MA, et al. Low-dose methotrexate therapy for ocular inflammatory disease. Ophthalmology 1992; 99:1419.
- Wizemann AJ, Wizemann V. Therapeutic effects of short-term plasma exchange in endogenous uveitis. Am J Ophthalmol 1984; 97:565.
- Moon SJ, Misch DM. Intravitreal Bevacizumab for Macular Edema from Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis. Ophthalmic Surg Lasers Imaging 2010; :1.
- Cheema RA, Al-Askar E, Cheema HR. Infliximab therapy for idiopathic retinal vasculitis, aneurysm, and neuroretinitis syndrome. J Ocul Pharmacol Ther 2011; 27:407.
- Karagiannis D, Soumplis V, Georgalas I, Kandarakis A. Ranibizumab for idiopathic retinal vasculitis, aneurysms, and neuroretinitis: favorable results. Eur J Ophthalmol 2010; 20:792.
- Henderly DE, Genstler AJ, Smith RE, Rao NA. Changing patterns of uveitis. Am J Ophthalmol 1987; 103:131.
- Malinowski SM, Pulido JS, Folk JC. Long-term visual outcome and complications associated with pars planitis. Ophthalmology 1993; 100:818.
- Smith RE, Godfrey WA, Kimura SJ. Chronic cyclitis. I. Course and visual prognosis. Trans Am Acad Ophthalmol Otolaryngol 1973; 77:OP760.
- Smith RE, Godfrey WA, Kimura SJ. Complications of chronic cyclitis. Am J Ophthalmol 1976; 82:277.
- Aaberg TM, Cesarz TJ, Flickinger RR. Treatment of peripheral uveoretinitis by cryotherapy. Am J Ophthalmol 1973; 75:685.
- Wong VG. Immunosuppressive therapy of ocular inflammatory diseases. Arch Ophthalmol 1969; 81:628.
- Serna-Ojeda JC, Pedroza-Seres M. Treatment with immunosuppressive therapy in patients with pars planitis: experience of a reference centre in Mexico. Br J Ophthalmol 2014; 98:1503.
- Ryan SJ, Maumenee AE. Birdshot retinochoroidopathy. Am J Ophthalmol 1980; 89:31.
- Priem HA, Oosterhuis JA. Birdshot chorioretinopathy: clinical characteristics and evolution. Br J Ophthalmol 1988; 72:646.
- Feltkamp TE. Ophthalmological significance of HLA associated uveitis. Eye (Lond) 1990; 4 ( Pt 6):839.
- Rothova A, Ossewaarde-van Norel A, Los LI, Berendschot TT. Efficacy of low-dose methotrexate treatment in birdshot chorioretinopathy. Retina 2011; 31:1150.
- Rush RB, Goldstein DA, Callanan DG, et al. Outcomes of birdshot chorioretinopathy treated with an intravitreal sustained-release fluocinolone acetonide-containing device. Am J Ophthalmol 2011; 151:630.
- Elliot AJ. 30-year observation of patients with Eale's disease. Am J Ophthalmol 1975; 80:404.
- Bali T, Saxena S, Kumar D, Nath R. Response time and safety profile of pulsed oral methotrexate therapy in idiopathic retinal periphlebitis. Eur J Ophthalmol 2005; 15:374.
- Patwardhan SD, Azad R, Shah BM, Sharma Y. Role of intravitreal bevacizumab in Eales disease with dense vitreous hemorrhage: a prospective randomized control study. Retina 2011; 31:866.
- Balducci N, Savini G, Barboni P, et al. Hemorrhagic Occlusive Retinal Vasculitis After First Eye Cataract Surgery Without Subsequent Second Eye Involvement. Ophthalmic Surg Lasers Imaging Retina 2016; 47:764.
- Ehmann DS, Adam MK, Kasi SK, et al. HEMORRHAGIC OCCLUSIVE RETINAL VASCULITIS AND NONHEMORRHAGIC VASCULITIS AFTER UNCOMPLICATED CATARACT SURGERY WITH INTRACAMERAL VANCOMYCIN. Retin Cases Brief Rep 2016.
- Lenci LT, Chin EK, Carter C, et al. Ischemic Retinal Vasculitis Associated with Cataract Surgery and Intracameral Vancomycin. Case Rep Ophthalmol Med 2015; 2015:683194.
- CLINICAL CHARACTERISTICS
- Physical examination
- OCULAR SYNDROMES ASSOCIATED WITH RETINAL VASCULITIS
- Idiopathic retinal vasculitis
- - Treatment
- Pars planitis syndrome
- - Treatment
- Birdshot retinochoroidopathy
- - Treatment
- Eales' disease
- - Treatment
- Hemorrhagic occlusive retinal vasculitis
- - Treatment
- SUMMARY AND RECOMMENDATIONS