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Non-infectious Retinal Vasculitis

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Systemic Vasculitides: Current Status and Perspectives

Abstract

Retinal vasculitis is a common clinical feature in intermediate and posterior uveitis. It is defined as either clinical or angiographic evidence of vascular inflammation accompanied by intraocular inflammation. It may be a primary inflammation of the blood vessel or, more commonly, secondary to inflammation of adjacent structures such as the retina or the choroid. In most series, the most common systemic diseases associated with retinal vasculitis is Behçet’s disease. Primary vasculitides such as polyarteritis nodosa or ANCA-associated vasculitis are rarely associated with retinal vasculitis. The most common clinical findings in retinal vasculitis are vitritis and vascular sheathing. The retinal veins are more commonly involved than the arteries. Treatment of retinal vasculitis is aimed at improving vision as well as preventing complications caused either by the intraocular inflammation or from ischemia and involves both systemic immunomodulatory therapy as well as ocular treatment such as intravitreal steroids and anti-VEGF injections. With proper therapy the prognosis is good.

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References

  1. Hughes EH, Dick AD (2003) The pathology and pathogenesis of retinal vasculitis. Neuropathol Appl Neurobiol 29:325–340

    Google Scholar 

  2. Jabs DA, Nussenblatt RB, Rosenbaum JT (2005) Standardization of uveitis nomenclature working group. Standardization of uveitis nomenclature for reporting clinical data. Results of the first international workshop. Am J Ophthalmol 140:509–516

    Article  PubMed  Google Scholar 

  3. Herbort CP, Cimino L, Abu El-Asrar AM (2005) Ocular vasculitis: a multidisciplinary approach. Curr Opin Rheumatol 17:25–33

    Article  PubMed  Google Scholar 

  4. Walton RC, Ashmore ED (2003) Retinal vasculitis. Curr Opin Ophthalmol 14:409–413

    Google Scholar 

  5. Abu El-Asrar AM, Herbort CP, Tabbara KF (2005) Retinal vasculitis. Ocul Immunol Inflamm 13:415–433

    Article  PubMed  Google Scholar 

  6. Levy-Clarke GA, Nussenblatt R (2005) Retinal vasculitis. Int Ophthalmol Clin 45:99–113

    Article  PubMed  Google Scholar 

  7. Abu El-Asrar AM, Tabbara KF (2010) A clinical approach to the diagnosis of retinal vasculitis. Int Ophthalmol 30:149–173

    Article  Google Scholar 

  8. Gupta A, Gupta V, Herbort CP et al (2009) Uveitis, text and imaging. Jaypee Brothers Medical Publishers, New Delhi

    Google Scholar 

  9. Fenton RH, Easom HA (1964) Behcet’s syndrome. A histopathologic study of the eye. Arch Ophthalmol 72:71–81

    Article  CAS  PubMed  Google Scholar 

  10. Charteris DG, Champ C, Rosenthal AR et al (1992) Behcet’s disease: activated T lymphocytes in retinal perivasculitis. Br J Ophthalmol 76:499–501

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. George RK, Chan CC, Whitcup SM et al (1997) Ocular immunopathology of Behcet’s disease. Surv Ophthalmol 42:157–162

    Article  CAS  PubMed  Google Scholar 

  12. Frank KW, Weiss H (1983) Unusual clinical and histopathological findings in ocular sarcoidosis. Br J Ophthalmol 67:8–16

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Graham EM, Spalton DJ, Barnard RO et al (1985) Cerebral and retinal vascular changes in systemic lupus erythematosus. Ophthalmology 92:444–448

    Article  CAS  PubMed  Google Scholar 

  14. Vine AK, Barr CC (1984) Proliferative lupus retinopathy. Arch Ophthalmol 102:852–854

    Article  CAS  PubMed  Google Scholar 

  15. Arnold AC, Pepose JS, Hepler RS et al (1984) Retinal periphlebitis and retinitis in multiple sclerosis. I. Pathologic characteristics. Ophthalmology 91:255–262

    Article  CAS  PubMed  Google Scholar 

  16. Lucarelli MJ, Pepose JS, Arnold AC et al (1991) Immunopathologic features of retinal lesions in multiple sclerosis. Ophthalmology 98:1652–1656

    Google Scholar 

  17. Engell T, Jensen OA, Klinken L (1985) Periphlebitis retinae in multiple sclerosis. A histopathological study of two cases. Acta Ophthalmol 63:83–88

    Article  CAS  Google Scholar 

  18. Hughes EH, Dick AD (2003) The pathology and pathogenesis of retinal vasculitis. Neuropathol Appl Neurobiol 29:325–340

    Article  CAS  PubMed  Google Scholar 

  19. Shulman S, Kramer M, Amer R et al (2015) Characteristics and long-term outcome of patients with noninfectious retinal vasculitis. Retina. May 27. [Epub ahead of print]

    Google Scholar 

  20. Ali A, Ku JH, Suhler EB et al (2014) The course of retinal vasculitis. Br J Ophthalmol 98:785–789

    Article  PubMed  Google Scholar 

  21. Stanford MR, Graham E, Kasp E et al (1988) A longitudinal study of clinical and immunological findings in 52 patients with relapsing retinal vasculitis. Br J Ophthalmol 72:442–444

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Graham EM, Stanford MR et al (1989) A point prevalence study of 150 patients with idiopathic retinal vasculitis: 1. Diagnostic value of ophthalmological features. Br J Ophthalmol 73:714–721

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. George RK, Walton RC, Whitcup SM, Nussenblatt RB (1996) Primary retinal vasculitis. Systemic associations and diagnostic evaluation. Ophthalmology 103:384–389

    Google Scholar 

  24. Leder HA, Campbell JP, Sepah YJ et al (2013) Ultra-wide-field retinal imaging in the management of non-infectious retinal vasculitis. J Ophthalmic Inflamm Infect 3(1):30

    Article  PubMed  PubMed Central  Google Scholar 

  25. Saurabh K, Das RR, Biswas J, Kumar A (2011) Profile of retinal vasculitis in a tertiary eye care center in eastern India. Indian J Ophthalmol 59:297–301

    Article  PubMed  PubMed Central  Google Scholar 

  26. Rosenbaum JT, Ku J, Ali A et al (2012) Patients with retinal vasculitis rarely suffer from systemic vasculitis. Semin Arthritis Rheum 41:859–865

    Article  PubMed  Google Scholar 

  27. Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA (1983) Ocular complications of Wegener’s granulomatosis. Ophthalmology 90:279–290

    Article  CAS  PubMed  Google Scholar 

  28. Rothschild PR, Pagnoux C, Seror R et al (2013) Ophthalmologic manifestations of systemic necrotizing vasculitides at diagnosis: a retrospective study of 1286 patients and review of the literature. Semin Arthritis Rheum 42:507–514

    Article  PubMed  Google Scholar 

  29. Kac¸maz RO, Kempen JH, Newcomb C et al (2008) Ocular inflammation in Behc¸et disease: incidence of ocular complications and of loss of visual acuity. Am J Opthalmol 146:828–836

    Article  Google Scholar 

  30. Verity DH, Vaughan GR, Stanford MR (2003) Behc¸et’s disease: from hippocrates to the third millennium. Br J Ophthalmol 87:1175–1183

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Taylor SRJ, Singh J, Menezo V et al (2011) Behet disease: visual prognosis and factors influencing the development of visual loss. Am J Ophthalmol 152:1059–1066

    Article  PubMed  Google Scholar 

  32. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R et al (2004) Uveitis in Behc¸et disease: an analysis of 880 patients. Am J Ophthalmol 138:373–380

    Article  PubMed  Google Scholar 

  33. Khalatbari D, Stinnett S, McCallum RM, Jaffe GJ (2004) Demographic-related variations in posterior segment ocular sarcoidosis. Ophthalmology 111:357–362

    Article  PubMed  Google Scholar 

  34. Herbort CP, Rao NA, Mochizuki M (2009) International criteria for the diagnosis of ocular sarcoidosis: results of the first international workshop on ocular sarcoidosis (IWOS). Ocul Immunol Inflamm 17:160–169

    Article  CAS  PubMed  Google Scholar 

  35. Graham EM, Francis DA, Sanders MD, Rudge P (1989) Ocular inflammatory changes in established multiple sclerosis. J Neurol Neurosurg Psychiatry 52:1360–1363

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. Towler HM, Lightman S (2000) Symptomatic intraocular inflammation in multiple sclerosis. Clin Exp Ophthalmol 28:97–102

    Article  CAS  PubMed  Google Scholar 

  37. Klinkhoff AV, Beattie CW, Chalmers A (1986) Retinopathy in systemic lupus erythematosus: relationship to disease activity. Arthritis Rheum 29:1152–1156

    Article  CAS  PubMed  Google Scholar 

  38. Belmont HM, Abramson SB, Lie JT (1996) Pathology and pathogenesis of vascular injury in systemic lupus erythematosus interactions of inflammatory cells and activated endothelium. Arthritis Rheum 39:9–22

    Article  CAS  PubMed  Google Scholar 

  39. Masuda K, Nakajima A, Urayama A et al (1989) Double-masked trial of cyclosporin versus colchicine and long-term open study of cyclosporin in Behcet’s disease. Lancet 1:1093–1096

    Article  CAS  PubMed  Google Scholar 

  40. Saadoun B, Wechsler B, Terrada C et al (2010) Azathioprine in severe uveitis of Behc¸et’s disease. Arthritis Care Res 62:1733–1738

    Article  CAS  Google Scholar 

  41. Baughman RP, Lower EE, Ingledue R, Kaufman AH (2012) Management of ocular sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 29:26–33

    CAS  PubMed  Google Scholar 

  42. Palejwala NV, Walia HS, Yeh S (2012) Ocular manifestations of systemic lupus erythematosus: a review of the literature. Autoimmune Dis 2012:29089

    Google Scholar 

  43. Levy-Clarke G, Jabs DA, Read RW et al (2013) Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology 121:785.e3–796.e3

    Google Scholar 

  44. Al Rashidi SA, Al Fawaz A, Kangave D, Abu El-Asrar AM (2013) Long-term clinical outcomes in patients with refractory uveitis associated with Behc¸et disease treated with infliximab. Ocul Immunol Inflamm 21:468–474

    Article  PubMed  Google Scholar 

  45. Bawazeer A, Raffa LH, Nizamuddin N (2010) Clinical experience with adalimumab in the treatment of ocular Behc¸et disease. Ocul Immunol Inflamm 18:226–232

    Article  CAS  PubMed  Google Scholar 

  46. Petropoulos IK, Vaudaux JD, Guex-Crosier Y (2008) AntiTNF-α therapy in patients with chronic non-infectious uveitis: the experience of Jules Gonin Eye Hospital. Klin Monatsbl 225:457–461

    Google Scholar 

  47. Erckens RJ, Mostard RLM, Wijnen AHM et al (2012) Adalimumab successful in sarcoidosis patients with refractory chronic non-infectious uveitis. Graefes Arch Clin Exp Ophthalmol 250:713–720

    Google Scholar 

  48. Cruz BA, Reis DD, Araujo CAA (2007) Refractory retinal vasculitis due to sarcoidosis successfully treated with infliximab. Rheumatol Int 27:1181–1183

    Article  CAS  PubMed  Google Scholar 

  49. Mohan M, Edwards ET, Cupps TR et al (2001) Demyelination occurring during anti-tumor necrosis factor alpha therapy for inflammatory arthritides. Arthritis Rheum 44:2862–2869

    Article  CAS  PubMed  Google Scholar 

  50. Kotter I, Eckstein AK, St¨ ubiger N, Zierhut M (1998) Treatment of ocular symptoms of Behc¸et’s disease with interferon alpha 2a: a pilot study. Br J Ophthalmol 82:488–494

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Kurup S, Lew J, Byrnes G et al (2009) Therapeutic efficacy of intravitreal bevacizumab on posterior uveitis complicated by neovascularization. Acta Ophthalmol 87:349–352

    Article  PubMed  Google Scholar 

  52. Lee WJ, Cho HY, Lee YJ et al (2011) Intravitreal bevacizumab for severe vaso-occlusive retinopathy in systemic lupus erythematosus. Rheumatol Int 33:247–251

    Article  PubMed  Google Scholar 

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Correspondence to Shiri Shulman .

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Shulman, S., Habot-Wilner, Z. (2016). Non-infectious Retinal Vasculitis. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_26

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