Retinal Vasculitis: Fundamentals, Diagnostics, and Management
DOI:
https://doi.org/10.33192/Smj.2021.64Keywords:
Retinal vasculitis, retinal vascular inflammation, posterior uveitisAbstract
Retinal vasculitis is inflammation of retinal blood vessels typically resulting from infection or immune-mediated inflammatory processes. It may present as isolated ocular inflammation or as a part of severe or potentially fatal systemic disease. Ocular complications of retinal vasculitis include cystoid macular edema, neovascularization, tractional retinal detachment, and vitreous hemorrhage, which all greatly threaten vision. Multimodal imaging and thorough systemic investigations are the main tools for making a precise diagnosis, which aids in predicting disease prognosis and visual outcome as well as preserving a patient’s vision and possibly their life. This review aims to discuss the current understanding of retinal vasculitis as well as current diagnostic tools and treatments.
References
1. Levy-Clarke GA, Nussenblatt R. Retinal vasculitis. Int Ophthalmol Clin. 2005;45(2):99-113.
2. Abu El-Asrar AM, Herbort CP, Tabbara KF. Retinal vasculitis. Ocul Immunol Inflamm. 2005;13(6):415-33.
3. Rosenbaum JT, Sibley CH, Lin P. Retinal vasculitis. Curr Opin Rheumatol. 2016;28(3):228-35.
4. Sharief L, Lightman S, Blum-Hareuveni T, Bar A, Tomkins-Netzer O. Clinical Outcome of Retinal Vasculitis and Predictors for Prognosis of Ischemic Retinal Vasculitis. Am J Ophthalmol. 2017;177:206-12.
5. Hughes EH, Dick AD. The pathology and pathogenesis of retinal vasculitis. Neuropathol Appl Neurobiol. 2003;29(4):325-40.
6. Davatchi F, Chams-Davatchi C, Shams H, Shahram F, Nadji A, Akhlaghi M, et al. Behcet's disease: epidemiology, clinical manifestations, and diagnosis. Expert Rev Clin Immunol. 2017;13(1):57-65.
7. Papagiannuli E, Rhodes B, Wallace GR, Gordon C, Murray PI, Denniston AK. Systemic lupus erythematosus: An update for ophthalmologists. Surv Ophthalmol. 2016;61(1):65-82.
8. Read RW. Clinical mini-review: systemic lupus erythematosus and the eye. Ocul Immunol Inflamm. 2004;12(2):87-99.
9. Albert DM, Raven ML. Ocular Tuberculosis. Microbiol Spectr. 2016;4(6):10.1128/microbiolspec.TNMI7-0001-2016.
10. Stanford MR, Graham EM, Kasp E, Brown EC, Dumonde DC, Sanders MD. Retinal vasculitis: correlation of animal and human disease. Eye (Lond). 1987;1( Pt 1):69-77.
11. LeHoang P, Sterkers M, Thillaye B, de Kozak Y, Coscas G, Faure JP. Primate model of uveoretinitis and vasculitis/experimental autoimmune uveoretinitis induced in cynomolgus monkeys by retinal s antigen. Ophthalmic Res. 2008;40(3-4):181-8.
12. Stanford MR, Brown EC, Kasp E, Graham EM, Sanders MD, Dumonde DC. Experimental posterior uveitis. I: A clinical, angiographic, and pathological study. Br J Ophthalmol. 1987;71(8):585-92.
13. Bassuk AG, Yeh S, Wu S, Martin DF, Tsang SH, Gakhar L, et al. Structural modeling of a novel CAPN5 mutation that causes uveitis and neovascular retinal detachment. PLoS One. 2015;10(4):e0122352.
14. Lee-Kirsch MA, Gong M, Chowdhury D, Senenko L, Engel K, Lee YA, et al. Mutations in the gene encoding the 3'-5' DNA exonuclease TREX1 are associated with systemic lupus erythematosus. Nat Genet. 2007;39(9):1065-7.
15. Zhou Q, Wang H, Schwartz DM, Stoffels M, Park YH, Zhang Y, et al. Loss-of-function mutations in TNFAIP3 leading to A20 haploinsufficiency cause an early-onset autoinflammatory disease. Nat Genet. 2016;48(1):67-73.
16. Abu El-Asrar AM, Herbort CP, Tabbara KF. Differential diagnosis of retinal vasculitis. Middle East Afr J Ophthalmol. 2009;16(4):202-18.
17. Foss AJ, Headon MP, Hamilton AM, Lightman S. Transient vessel wall sheathing in acute retinal vein occlusions. Eye (Lond). 1992;6(Pt 3):313-6.
18. Hayreh SS, Zimmerman MB. Fundus changes in branch retinal vein occlusion. Retina. 2015;35(5):1016-27.
19. Grosso A, Pellegrini M, Cereda MG, Panico C, Staurenghi G, Sigler EJ. Pearls and pitfalls in diagnosis and management of coats disease. Retina. 2015;35(4):614-23.
20. Maleki A, Cao JH, Silpa-Archa S, Foster CS. Visual Outcome and Poor Prognostic Factors in Isolated Idiopathic Retinal Vasculitis. Retina. 2016;36(10):1979-85.
21. Agarwal A, Afridi R, Agrawal R, Do DV, Gupta V, Nguyen QD. Multimodal Imaging in Retinal Vasculitis. Ocul Immunol Inflamm. 2017;25(3):424-33.
22. Nicholson BP, Nigam D, Miller D, Agron E, Dalal M, Jacobs-El N, et al. Comparison of wide-field fluorescein angiography and 9-field montage angiography in uveitis. Am J Ophthalmol. 2014;157(3):673-7.
23. Sheemar A, Temkar S, Takkar B, Sood R, Sinha S, Chawla R, et al. Ultra-Wide Field Imaging Characteristics of Primary Retinal Vasculitis: Risk Factors for Retinal Neovascularization. Ocul Immunol Inflamm. 2019;27(3):383-8.
24. Jones NP, Sala-Puigdollers A, Stanga PE. Ultra-widefield fundus fluorescein angiography in the diagnosis and management of retinal vasculitis. Eye (Lond). 2017;31(11):1546-9.
25. Mahendradas P, Vala R, Kawali A, Akkali MC, Shetty R. Adaptive Optics Imaging in Retinal Vasculitis. Ocul Immunol Inflamm. 2018;26(5):760-6.
26. Spaide RF. Microvascular flow abnormalities associated with retinal vasculitis: A Potential of Mechanism of Retinal Injury. Retina. 2017;37(6):1034-42.
27. Sallam A, Taylor SR, Lightman S. Review and update of intraocular therapy in noninfectious uveitis. Curr Opin Ophthalmol. 2011;22(6):517-22.
28. Shah KK, Majumder PD, Biswas J. Intravitreal therapeutic agents in noninfectious uveitic macular edema. Indian J Ophthalmol. 2018;66(8):1060-73.
29. Thorne JE, Sugar EA, Holbrook JT, Burke AE, Altaweel MM, Vitale AT, et al. Periocular Triamcinolone vs. Intravitreal Triamcinolone vs. Intravitreal Dexamethasone Implant for the Treatment of Uveitic Macular Edema: The PeriOcular vs. INTravitreal corticosteroids for uveitic macular edema (POINT) Trial. Ophthalmology. 2019;126(2):283-95.
30. Couret C, Poinas A, Volteau C, Riche VP, Le Lez ML, Errera MH, et al. Comparison of two techniques used in routine care for the treatment of inflammatory macular oedema, subconjunctival triamcinolone injection and intravitreal dexamethasone implant: medical and economic importance of this randomized controlled trial. Trials. 2020;21(1):159.
31. Phulke S, Kaushik S, Kaur S, Pandav SS. Steroid-induced Glaucoma: An Avoidable Irreversible Blindness. J Curr Glaucoma Pract. 2017;11(2):67-72.
32. Jinagal J, Gupta PC, Pilania RK, Ram J. Systemic toxicity of topical corticosteroids. Indian J Ophthalmol. 2019;67(4):559-61.
33. Kacmar J, Cholevik D. Corticosteroid Induced Posterior Subcapsular Cataract. Cesk Slov Oftalmol. 2019;74(6):226-32.
34. Ton FN, Gunawardene SC, Lee H, Neer RM. Effects of low-dose prednisone on bone metabolism. J Bone Miner Res. 2005;20(3):464-70.
35. Jabs DA, Rosenbaum JT, Foster CS, Holland GN, Jaffe GJ, Louie JS, et al. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol. 2000;130(4):492-513.
36. Foster CS, Kothari S, Anesi SD, Vitale AT, Chu D, Metzinger JL, et al. The Ocular Immunology and Uveitis Foundation preferred practice patterns of uveitis management. Surv Ophthalmol. 2016;61(1):1-17.
37. Talat L, Lightman S, Tomkins-Netzer O. Ischemic retinal vasculitis and its management. J Ophthalmol. 2014;2014:197675.
38. Jabs DA. Immunosuppression for the Uveitides. Ophthalmology. 2018;125(2):193-202.
39. Levy-Clarke G, Jabs DA, Read RW, Rosenbaum JT, Vitale A, Van Gelder RN. Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology. 2014;121(3):785-96 e3.
40. Goto H, Zako M, Namba K, Hashida N, Kaburaki T, Miyazaki M, et al. Adalimumab in Active and Inactive, Non-Infectious Uveitis: Global Results from the VISUAL I and VISUAL II Trials. Ocul Immunol Inflamm. 2019;27(1):40-50.
41. Lin M, Anesi SD, Ma L, Ahmed A, Small K, Foster CS. Characteristics and Visual Outcome of Refractory Retinal Vasculitis Associated With Antineutrophil Cytoplasm Antibody-Associated Vasculitides. Am J Ophthalmol. 2018;187:21-33.
42. Tallouzi MO, Moore DJ, Barry RJ, Calvert M, Mathers J, Murray PI, et al. The Effectiveness of Pharmacological Agents for the Treatment of Uveitic Macular Edema (UMO): A Systematic Review. Ocul Immunol Inflamm. 2019;27(4):658-80.
43. Rouvas A, Nikita E, Markomichelakis N, Theodossiadis P, Pharmakakis N. Idiopathic retinal vasculitis, arteriolar macroaneurysms and neuroretinitis: clinical course and treatment. J Ophthalmic Inflamm Infect. 2013;3(1):21.
44. Ali A, Ku JH, Suhler EB, Choi D, Rosenbaum JT. The course of retinal vasculitis. Br J Ophthalmol. 2014;98(6):785-9.
2. Abu El-Asrar AM, Herbort CP, Tabbara KF. Retinal vasculitis. Ocul Immunol Inflamm. 2005;13(6):415-33.
3. Rosenbaum JT, Sibley CH, Lin P. Retinal vasculitis. Curr Opin Rheumatol. 2016;28(3):228-35.
4. Sharief L, Lightman S, Blum-Hareuveni T, Bar A, Tomkins-Netzer O. Clinical Outcome of Retinal Vasculitis and Predictors for Prognosis of Ischemic Retinal Vasculitis. Am J Ophthalmol. 2017;177:206-12.
5. Hughes EH, Dick AD. The pathology and pathogenesis of retinal vasculitis. Neuropathol Appl Neurobiol. 2003;29(4):325-40.
6. Davatchi F, Chams-Davatchi C, Shams H, Shahram F, Nadji A, Akhlaghi M, et al. Behcet's disease: epidemiology, clinical manifestations, and diagnosis. Expert Rev Clin Immunol. 2017;13(1):57-65.
7. Papagiannuli E, Rhodes B, Wallace GR, Gordon C, Murray PI, Denniston AK. Systemic lupus erythematosus: An update for ophthalmologists. Surv Ophthalmol. 2016;61(1):65-82.
8. Read RW. Clinical mini-review: systemic lupus erythematosus and the eye. Ocul Immunol Inflamm. 2004;12(2):87-99.
9. Albert DM, Raven ML. Ocular Tuberculosis. Microbiol Spectr. 2016;4(6):10.1128/microbiolspec.TNMI7-0001-2016.
10. Stanford MR, Graham EM, Kasp E, Brown EC, Dumonde DC, Sanders MD. Retinal vasculitis: correlation of animal and human disease. Eye (Lond). 1987;1( Pt 1):69-77.
11. LeHoang P, Sterkers M, Thillaye B, de Kozak Y, Coscas G, Faure JP. Primate model of uveoretinitis and vasculitis/experimental autoimmune uveoretinitis induced in cynomolgus monkeys by retinal s antigen. Ophthalmic Res. 2008;40(3-4):181-8.
12. Stanford MR, Brown EC, Kasp E, Graham EM, Sanders MD, Dumonde DC. Experimental posterior uveitis. I: A clinical, angiographic, and pathological study. Br J Ophthalmol. 1987;71(8):585-92.
13. Bassuk AG, Yeh S, Wu S, Martin DF, Tsang SH, Gakhar L, et al. Structural modeling of a novel CAPN5 mutation that causes uveitis and neovascular retinal detachment. PLoS One. 2015;10(4):e0122352.
14. Lee-Kirsch MA, Gong M, Chowdhury D, Senenko L, Engel K, Lee YA, et al. Mutations in the gene encoding the 3'-5' DNA exonuclease TREX1 are associated with systemic lupus erythematosus. Nat Genet. 2007;39(9):1065-7.
15. Zhou Q, Wang H, Schwartz DM, Stoffels M, Park YH, Zhang Y, et al. Loss-of-function mutations in TNFAIP3 leading to A20 haploinsufficiency cause an early-onset autoinflammatory disease. Nat Genet. 2016;48(1):67-73.
16. Abu El-Asrar AM, Herbort CP, Tabbara KF. Differential diagnosis of retinal vasculitis. Middle East Afr J Ophthalmol. 2009;16(4):202-18.
17. Foss AJ, Headon MP, Hamilton AM, Lightman S. Transient vessel wall sheathing in acute retinal vein occlusions. Eye (Lond). 1992;6(Pt 3):313-6.
18. Hayreh SS, Zimmerman MB. Fundus changes in branch retinal vein occlusion. Retina. 2015;35(5):1016-27.
19. Grosso A, Pellegrini M, Cereda MG, Panico C, Staurenghi G, Sigler EJ. Pearls and pitfalls in diagnosis and management of coats disease. Retina. 2015;35(4):614-23.
20. Maleki A, Cao JH, Silpa-Archa S, Foster CS. Visual Outcome and Poor Prognostic Factors in Isolated Idiopathic Retinal Vasculitis. Retina. 2016;36(10):1979-85.
21. Agarwal A, Afridi R, Agrawal R, Do DV, Gupta V, Nguyen QD. Multimodal Imaging in Retinal Vasculitis. Ocul Immunol Inflamm. 2017;25(3):424-33.
22. Nicholson BP, Nigam D, Miller D, Agron E, Dalal M, Jacobs-El N, et al. Comparison of wide-field fluorescein angiography and 9-field montage angiography in uveitis. Am J Ophthalmol. 2014;157(3):673-7.
23. Sheemar A, Temkar S, Takkar B, Sood R, Sinha S, Chawla R, et al. Ultra-Wide Field Imaging Characteristics of Primary Retinal Vasculitis: Risk Factors for Retinal Neovascularization. Ocul Immunol Inflamm. 2019;27(3):383-8.
24. Jones NP, Sala-Puigdollers A, Stanga PE. Ultra-widefield fundus fluorescein angiography in the diagnosis and management of retinal vasculitis. Eye (Lond). 2017;31(11):1546-9.
25. Mahendradas P, Vala R, Kawali A, Akkali MC, Shetty R. Adaptive Optics Imaging in Retinal Vasculitis. Ocul Immunol Inflamm. 2018;26(5):760-6.
26. Spaide RF. Microvascular flow abnormalities associated with retinal vasculitis: A Potential of Mechanism of Retinal Injury. Retina. 2017;37(6):1034-42.
27. Sallam A, Taylor SR, Lightman S. Review and update of intraocular therapy in noninfectious uveitis. Curr Opin Ophthalmol. 2011;22(6):517-22.
28. Shah KK, Majumder PD, Biswas J. Intravitreal therapeutic agents in noninfectious uveitic macular edema. Indian J Ophthalmol. 2018;66(8):1060-73.
29. Thorne JE, Sugar EA, Holbrook JT, Burke AE, Altaweel MM, Vitale AT, et al. Periocular Triamcinolone vs. Intravitreal Triamcinolone vs. Intravitreal Dexamethasone Implant for the Treatment of Uveitic Macular Edema: The PeriOcular vs. INTravitreal corticosteroids for uveitic macular edema (POINT) Trial. Ophthalmology. 2019;126(2):283-95.
30. Couret C, Poinas A, Volteau C, Riche VP, Le Lez ML, Errera MH, et al. Comparison of two techniques used in routine care for the treatment of inflammatory macular oedema, subconjunctival triamcinolone injection and intravitreal dexamethasone implant: medical and economic importance of this randomized controlled trial. Trials. 2020;21(1):159.
31. Phulke S, Kaushik S, Kaur S, Pandav SS. Steroid-induced Glaucoma: An Avoidable Irreversible Blindness. J Curr Glaucoma Pract. 2017;11(2):67-72.
32. Jinagal J, Gupta PC, Pilania RK, Ram J. Systemic toxicity of topical corticosteroids. Indian J Ophthalmol. 2019;67(4):559-61.
33. Kacmar J, Cholevik D. Corticosteroid Induced Posterior Subcapsular Cataract. Cesk Slov Oftalmol. 2019;74(6):226-32.
34. Ton FN, Gunawardene SC, Lee H, Neer RM. Effects of low-dose prednisone on bone metabolism. J Bone Miner Res. 2005;20(3):464-70.
35. Jabs DA, Rosenbaum JT, Foster CS, Holland GN, Jaffe GJ, Louie JS, et al. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol. 2000;130(4):492-513.
36. Foster CS, Kothari S, Anesi SD, Vitale AT, Chu D, Metzinger JL, et al. The Ocular Immunology and Uveitis Foundation preferred practice patterns of uveitis management. Surv Ophthalmol. 2016;61(1):1-17.
37. Talat L, Lightman S, Tomkins-Netzer O. Ischemic retinal vasculitis and its management. J Ophthalmol. 2014;2014:197675.
38. Jabs DA. Immunosuppression for the Uveitides. Ophthalmology. 2018;125(2):193-202.
39. Levy-Clarke G, Jabs DA, Read RW, Rosenbaum JT, Vitale A, Van Gelder RN. Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology. 2014;121(3):785-96 e3.
40. Goto H, Zako M, Namba K, Hashida N, Kaburaki T, Miyazaki M, et al. Adalimumab in Active and Inactive, Non-Infectious Uveitis: Global Results from the VISUAL I and VISUAL II Trials. Ocul Immunol Inflamm. 2019;27(1):40-50.
41. Lin M, Anesi SD, Ma L, Ahmed A, Small K, Foster CS. Characteristics and Visual Outcome of Refractory Retinal Vasculitis Associated With Antineutrophil Cytoplasm Antibody-Associated Vasculitides. Am J Ophthalmol. 2018;187:21-33.
42. Tallouzi MO, Moore DJ, Barry RJ, Calvert M, Mathers J, Murray PI, et al. The Effectiveness of Pharmacological Agents for the Treatment of Uveitic Macular Edema (UMO): A Systematic Review. Ocul Immunol Inflamm. 2019;27(4):658-80.
43. Rouvas A, Nikita E, Markomichelakis N, Theodossiadis P, Pharmakakis N. Idiopathic retinal vasculitis, arteriolar macroaneurysms and neuroretinitis: clinical course and treatment. J Ophthalmic Inflamm Infect. 2013;3(1):21.
44. Ali A, Ku JH, Suhler EB, Choi D, Rosenbaum JT. The course of retinal vasculitis. Br J Ophthalmol. 2014;98(6):785-9.
Published
01-08-2021
How to Cite
Boonsopon, S. ., & Stephen D. Anesi. (2021). Retinal Vasculitis: Fundamentals, Diagnostics, and Management . Siriraj Medical Journal, 73(8), 493–500. https://doi.org/10.33192/Smj.2021.64
Issue
Section
Review Article
License
Users are free to share, copy, and redistribute all articles published in the Siriraj Medical Journal (SMJ) in any medium or format as long as you follow the following terms:
- Attribution — You must give appropriate credit, provide a link to the material, and indicate if changes were made. You may do so in any reasonable manner, but not in any way that suggests the publisher endorses you or your use.
- NonCommercial — You may not use the material for commercial purposes.
- NoDerivatives — If you remix, transform, or build upon the material, you may not distribute the modified material.
- No additional restrictions — You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.
