Abstract
Retinal vasculitis is a sight-threatening inflammatory eye condition that involves the retinal vessels. Detection of retinal vasculitis is made clinically, and is confirmed with the help of fundus fluorescein angiography. Active vascular disease is characterized by exudates around retinal vessels resulting in white sheathing or cuffing of the affected vessels. In this review, a practical approach to the diagnosis of retinal vasculitis is discussed based on ophthalmoscopic and fundus fluorescein angiographic findings.



























Similar content being viewed by others
References
Abu El-Asrar AM, Herbort CP, Tabbara KF (2005) Retinal vasculitis. Ocul Immunol Inflamm 13:415–433. doi:10.1080/09273940591003828
Arnold AC, Usaf M, Pepose JS, Hepler RS, Foos RY (1984) Retinal periphlebitis and retinitis in multiple sclerosis. I. Pathologic characteristics. Ophthalmology 91:255–262
Gass JD, Olson CL (1976) Sarcoidosis with optic nerve and retinal involvement. Arch Ophthalmol 94:945–950
Charteris DG, Champ C, Rosenthal AR, Lightman SL (1992) Behçet’s disease: activated T lymphocytes in retinal perivasculitis. Br J Ophthalmol 76:499–501. doi:10.1136/bjo.76.8.499
Pederson JE, Kenyon KR, Green WR, Maumenee AE (1978) Pathology of pars planitis. Am J Ophthalmol 86:762–774
Gaudio PA, Kaye DB, Crawford JB (2002) Histopathology of birdshot retinochoroidopathy. Br J Ophthalmol 86:1439–1441. doi:10.1136/bjo.86.12.1439
Perez VL, Chavala SH, Ahmed M, Chu D, Zafirakis P, Baltatzis S, Ocampo V, Foster CS (2004) Ocular manifestations and concepts of systemic vasculitides. Surv Ophthalmol 49:399–418. doi:10.1016/j.survophthal.2004.04.008
Graham EM, Spalton DJ, Barnard RO, Garner A, Russell RW (1985) Cerebral and retinal vascular changes in systemic lupus erythematosus. Ophthalmology 92:444–448
Au A, O’Day J (2004) Review of severe vaso-occlusive retinopathy in systemic lupus erythematosus and the antiphosopholipid syndrome: associations, visual outcomes, complications and treatment. Clin Exp Ophthalmol 32:87–100. doi:10.1046/j.1442-9071.2004.00766.x
Palmer HE, Stanford MR, Sanders MD, Graham EM (1996) Visual outcome of patients with idiopathic ischaemic and non-ischaemic retinal vasculitis. Eye 10:343–348
Saatci OA, Koçak N, Durak I, Ergin MH (2001) Unilateral retinal vasculitis, branch retinal artery occlusion and subsequent retinal neovascularization in Crohn’s disease. Int Ophthalmol 24:89–92. doi:10.1023/A:1016351800466
Abu El-Asrar AM, Naddaf HO, Al-Momen A, Al-Balla SR (1995) Systemic lupus erythematosus flare-up manifesting as a cilioretinal artery occlusion. Lupus 4:158–160. doi:10.1177/096120339500400216
Iida T, Spaide RF, Kantor J (2002) Retinal and choroidal arterial occlusion in Wegener’s granulomatosis. Am J Ophthalmol 133:151–152. doi:10.1016/S0002-9394(01)01251-X
Hsu CT, Kerrison JB, Miller NR, Goldberg MF (2001) Choroidal infarction, ischemic optic neuropathy, and central retinal artery occlusion from polyarteritis nodosa. Retina 21:348–351. doi:10.1097/00006982-200108000-00009
Skrapari I, Kagkelari E, Charitatos E, Pantelidaki C, Gounaris T, Sioula E (2008) Acute painless monocular visual loss due to central retinal artery occlusion in a patient with Churg–Strauss vasculitis. Clin Rheumatol 27:125–127. doi:10.1007/s10067-007-0695-x
Johnson MW, Thomley ML, Huang SS, Gass JDM (1994) Idiopathic recurrent branch retinal arterial occlusion. Natural history and laboratory evaluation. Ophthalmology 101:480–489
Emad Y, Basaffar S, Ragab Y, Zeinhom F, Gheita T (2007) A case of polyarteritis nodosa complicated by left central retinal artery occlusion, ischemic optic neuropathy, and retinal vasculitis. Clin Rheumatol 26:814–816. doi:10.1007/s10067-006-0270-x
Bentley CR, Stanford MR, Shilling JS, Sanders MD, Graham EM (1993) Macular ischaemia in posterior uveitis. Eye 7:411–414
Abu El-Asrar AM, AL-Kharashi SA (2002) Full panretinal photocoagulation and early vitrectomy improve prognosis of retinal vasculitis associated with tuberculoprotein hypersensitivity. Br J Ophthalmol 86:1248–1251. doi:10.1136/bjo.86.11.1248
Vine AK (1992) Severe periphlebitis, peripheral retinal ischemia, and preretinal neovascularization in patients with multiple sclerosis. Am J Ophthalmol 113:28–32
Morse PH (1975) Retinal venous sheathing and neovascularization in disseminated sclerosis. Ann Ophthalmol 7:949–952
Valentincic NV, Kraut A, Rothova A (2007) Vitreous hemorrhage in multiple sclerosis-associated uveitis. Ocul Immunol Inflamm 15:19–25. doi:10.1080/09273940601147737
Jabs DA, Fine SL, Hochberg MC, Newman SA, Heiner GG, Stevens MB (1986) Severe retinal vaso-occlusive disease in systemic lupus erythematosus. Arch Ophthalmol 104:558–563
Salmon JF, Ursell PG, Frith P (2000) Neovascular glaucoma as a complication of retinal vasculitis in Crohn’s disease. Am J Ophthalmol 130:528–530. doi:10.1016/S0002-9394(00)00609-7
Abu El-Asrar AM, Al-Obeidan SA, Abdel Gader AGM (2003) Retinal periphlebitis resembling frosted branch angiitis with nonperfused central retinal vein occlusion. Eur J Ophthalmol 13:807–812
Sakane T, Takeno M (2000) Novel approaches to Behçet’s disease. Expert Opin Investig Drugs 9:1993–2005. doi:10.1517/13543784.9.9.1993
Okada AA (2000) Drug therapy in Behçet’s disease. Ocul Immunol Inflamm 8:85–91. doi:10.1076/0927-3948(200006)8:2;1-0;FT085
Graham EM, Stanford MR, Sander MD, Kasp E, Dumonde DC (1989) A point prevalence study of 150 patients with idiopathic retinal vasculitis: I. Diagnostic value of ophthalmological features. Br J Ophthalmol 73:714–721. doi:10.1136/bjo.73.9.714
Gold DH, Morris DA, Hertrind P (1972) Ocular findings in systemic lupus erythematosus. Br J Ophthalmol 56:800–804. doi:10.1136/bjo.56.11.800
Lanham JG, Barrie T, Kohner EM, Hughes GRVO (1982) SLE retinopathy: evaluation by fluorescein angiography. Ann Rheum Dis 41:473–478. doi:10.1136/ard.41.5.473
Asherson RA, Merry P, Acheson JF, Harris EN, Hughes GRV (1989) Antiphospholipid antibodies: a risk factor for occlusive ocular vascular disease in systemic lupus erythematosus and the primary antiphospholipid syndrome. Ann Rheum Dis 48:358–361. doi:10.1136/ard.48.5.358
Quillen DA, Stathopoulos NA, Blankenship GW, Ferriss JA (1997) Lupus associated frosted branch periphlebitis and exudative maculopathy. Retina 17:449–451. doi:10.1097/00006982-199717050-00016
Karpik AG, Schwartz MM, Dickey LE, Streeten BW, Roberts JL (1985) Ocular immune reactants in patients with systemic lupus erythematosus. Clin Immunol Immunopathol 35:295–312. doi:10.1016/0090-1229(85)90091-1
Morgan CM, Foster CS, Gragoudas ES (1986) Retinal vasculitis in polyarteritis nodosa. Retina 6:205–209. doi:10.1097/00006982-198606040-00002
Akova YA, Jabbur NS, Foster CS (1993) Ocular presentation of polyarteritis nodosa. Clinical course and management with steroid and cytotoxic therapy. Ophthalmology 100:1775–1781
Curi ALL, Freeman G, Pavesio C (2001) Aggressive retinal vasculitis in polyarteritis nodosa. Eye 15:229–231
O’Halloran HS, Pearson PA, Lee WB, Susac JO, Berger JR (1998) Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature. Ophthalmology 105:1038–1044. doi:10.1016/S0161-6420(98)96005-5
Do TH, Fisch C, Evoy F (2004) Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol 25:382–388
Susac JO, Egan RA, Rennebohm RM, Lubow M (2007) Susac’s syndrome: 1975–2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci 257:270–272. doi:10.1016/j.jns.2007.01.036
Martinet N, Fardeau C, Adam R, Bodaghi B, Papo T, Piette JC, Lehoang P (2007) Fluorescein and indocyanine green angiographies in Susac syndrome. Retina 27:1242–1283
Theodossiodis P, Kokolakis S, Ladas I, Kollia AC, Chatzonlis D, Theodossiadis G (1995) Retinal vascular involvement in acute toxoplasmic retinochoroiditis. Int Ophthalmol 19:19–24. doi:10.1007/BF00156414
Ysasaga JE, Davis J (1999) Frosted branch angiitis with ocular toxoplasmosis. Arch Ophthalmol 117:1260–1261
Diaz-Valle D, Diaz-Rodriguez E, Diaz-Valle T, Benitez del Castillo JM, Toledano N, Fernandez Acenero MJ (2003) Frosted branch angiitis and late peripheral retinochoroidal scar in a patient with acquired toxoplasmosis. Eur J Ophthalmol 13:726–728
Holland GN, Muccioli C, Silveira C, Weisz JM, Belfort R Jr, O’Connor GR (1999) Intraocular inflammatory reactions without focal necrotizing retinochoroiditis in patients with acquired systemic toxoplasmosis. Am J Ophthalmol 128:413–420. doi:10.1016/S0002-9394(99)00300-1
Ganatra JB, Chandler D, Santos C, Kuppermann B, Margolis TP (2000) Viral causes of the acute retinal necrosis syndrome. Am J Ophthalmol 129:166–172. doi:10.1016/S0002-9394(99)00316-5
Holland GN (1994) Standard diagnostic criteria for the acute retinal necrosis syndrome. Am J Ophthalmol 117:663–667 Executive Committee of the American Uveitis Society
Yoser SL, Forster DJ, Rao NA (1993) Systemic viral infections and their retinal and choroidal manifestations. Surv Ophthalmol 37:313–352. doi:10.1016/0039-6257(93)90064-E
Culbertson WW (1989) Infections of the retina in AIDS. Int Ophthalmol Clin 29:108–118. doi:10.1097/00004397-198902920-00009
Spaide RF, Vitale AT, Toth IR, Oliver JM (1992) Frosted branch angiitis associated with cytomegalovirus retinitis. Am J Ophthalmol 113:522–528
Geier SA, Nasemann J, Klauss V, Kronawitter U, Goebel FD (1992) Frosted branch angiitis in a patient with the acquired immunodeficiency syndrome. Am J Ophthalmol 113:203–205
Kestelyn P, Lepage P, Van de Perre P (1985) Perivasculitis of the retinal vessels as an important sign in children with AIDS-related complex. Am J Ophthalmol 100:614–615
Kestelyn P, Van de Perre P, Rouvroy D, Lepage P, Bogaerts J, Nzaramba D, Clumeck N (1985) A prospective study of the ophthalmologic findings in the acquired immune deficiency syndrome in Africa. Am J Ophthalmol 100:230–238
Fine HF, Smith JA, Murante BL, Nussenblatt RB, Robinson MR (2001) Frosted branch angiitis in a child with HIV infection. Am J Ophthalmol 131:394–396. doi:10.1016/S0002-9394(00)00792-3
Sasaki K, Morooka I, Inomata H, Kashio N, Akamine T, Osame M (1989) Retinal vasculitis in human T-lymphotropic virus type 1 associated myelopathy. Br J Ophthalmol 73:812–815. doi:10.1136/bjo.73.10.812
Nakao K, Ohba N (1993) Clinical features of HTLV-1 associated uveitis. Br J Ophthalmol 77:274–279. doi:10.1136/bjo.77.5.274
Nakao K (1996) Ohba N. HTLV-1 associated uveitis revisited: Characteristic grey-white granular deposits on retinal vessels. Br J Ophthalmol 80:719–722. doi:10.1136/bjo.80.8.719
Nakao K, Ohba N (2003) Human T-cell lymphotropic virus type 1-associated retinal vasculitis in children. Retina 23:197–201. doi:10.1097/00006982-200304000-00010
Levy-Clarke GA, Buggage RR, Shen D, Vaughen LO, Chan CC, Davis JL (2002) Human T-cell lymphotropic virus-type 1 associated T-cell leukemia/lymphoma masquerading as necrotizing retinal vasculitis. Ophthalmology 109:1717–1722. doi:10.1016/S0161-6420(02)01132-6
Kincaid J, Schatz H (1983) Bilateral retinal arteritis with multiple aneurysmal dilatations. Retina 3:171–178. doi:10.1097/00006982-198300330-00005
Chang TS, Aylward W, Davis JL, Mieler WF, Oliver GL, Maberley AL, Gass JD (1995) Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis. Ophthalmology 102:1089–1097
Samuel MA, Equi RA, Chang TS, Mieler W, Jampol LM, Hay D, Yannuzzi LA (2007) Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN): new observations and a proposed staging system. Ophthalmology 114:1526–1529. doi:10.1016/j.ophtha.2006.11.014
Abu El-Asrar AM, Jestaneiah S, Al-Serhani AM (2004) Regression of aneurysmal dilatations in a case of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) associated with allergic fungal sinusitis. Eye 18:197–199. doi:10.1038/sj.eye.6700554
Owens SL, Gregor ZJ (1992) Vanishing retinal arterial aneurysms: a case report. Br J Ophthalmol 76:637–638. doi:10.1136/bjo.76.10.637
Sashihara H, Hayashi H, Oshima K (1999) Regression of retinal arterial aneurysms in a case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN). Retina 19:250–251
Tomita M, Matsubara T, Yamada H, Takahashi K, Nishimura T, Sho K, Uyama M, Matsumura M (2004) Long term follow up in a case of successfully treated idiopathic retinal vasculitis, aneurysms, and neuroretinitis. Br J Ophthalmol 88:302–303. doi:10.1136/bjo.2003.023002
Rothova A, Lardenoye C (1998) Arterial macroaneurysms in peripheral multifocal chorioretinitis associated with sarcoidosis. Ophthalmology 105:1393–1397. doi:10.1016/S0161-6420(98)98018-6
Verougstraete C, Snyers B, Leys A, Caspers-Velu LE (2001) Multiple arterial ectasias in patients with sarcoidosis and uveitis. Am J Ophthalmol 131:223–231. doi:10.1016/S0002-9394(00)00786-8
Yamanaka E, Ohguro N, Nubota A, Yamamoto S, Nakagawa Y, Tano Y (2004) Features of retinal arterial macroaneurysms in patients with uveitis. Br J Ophthalmol 88:884–886. doi:10.1136/bjo.2003.035923
Ito Y, Nakano M, Kyu N, Takeuchi M (1976) Frosted branch angiitis in a child. Jpn J Clin Ophthalmol 30:797–803
Kleiner RC, Kaplan HJ, Shakin JL, Yannuzzi LA, Crosswell HH Jr, McLean WC Jr (1988) Acute frosted retinal periphlebitis. Am J Ophthalmol 106:27–34. doi:10.1016/0002-9394(88)90365-0
Kleiner RC (1997) Frosted branch angiitis: clinical syndrome or clinical sign? Retina 17:370–371
Sykes SO, Horton JC (1997) Steroid-responsive retinal vasculitis with a frosted branch appearance in Crohn’s disease. Retina 17:451–454. doi:10.1097/00006982-199717050-00017
Markomichelakis NN, Barampouti F, Zafirakis P, Chalkiadakis I, Kouris T, Ekonomopoulos N (1999) Retinal vasculitis with a frosted branch angiitis-like response due to herpes simplex virus type 2. Retina 19:455–457. doi:10.1097/00006982-199919050-00019
Farrardo J, Fonollosa A, Segura A, Garcia-Arumi J (2008) Frosted branch angiitis associated with Epstein-Barr virus systemic infection. Ocul Immunol Inflamm 16:41–43. doi:10.1080/09273940701799114
Sugin SL, Henderly DE, Friedman SM, Jampol LM, Doyle JW (1991) Unilateral frosted branch angiitis. Am J Ophthalmol 111:682–685
Borkowski LM, Jampol LM (1999) Frosted branch angiitis complicated by retinal neovascularization. Retina 19:454–455. doi:10.1097/00006982-199919050-00018
Helm CJ, Holland GN (1993) Ocular tuberculosis. Surv Ophthalmol 38:229–256. doi:10.1016/0039-6257(93)90076-J
Rosen PH, Spalton DJ, Graham EM (1990) Intraocular tuberculosis. Eye 4:486–492
Gupta A, Gupta V, Arora S, Dogra MR, Bambery R (2001) PCR-positive tubercular retinal vasculitis. Clinical characteristics and management. Retina 21:435–444. doi:10.1097/00006982-200110000-00004
Biswas J, Sharma T, Gopal L, Madhavan HN, Sulochana KN, Ramakrishnan S (2002) Eales’ disease—an update. Surv Ophthalmol 47:197–214. doi:10.1016/S0039-6257(02)00288-6
International Study Group for Behçet’s Disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335:1078–1080
Verity DH, Wallace GR, Vaughan RW, Stanford MR (2003) Behçet’s disease: from Hippocrates to the third millennium. Br J Ophthalmol 87:1175–1183. doi:10.1136/bjo.87.9.1175
Lim JI, Tessler HH, Goodwin JA (1991) Anterior granulomatous uveitis in patients with multiple sclerosis. Ophthalmology 98:142–145
Graham EM, Francis DA, Sanders MD, Rudge P (1989) Ocular inflammatory changes in established multiple sclerosis. J Neurol Neurosurg Psychiatry 52:1360–1363. doi:10.1136/jnnp.52.12.1360
Engell T, Andersen PK (1982) The frequency of periphlebitis retinae in multiple sclerosis. Acta Neurol Scand 65:601–608
Birch MK, Barbosa S, Blumhardt LD, O’Brien C, Harding SP (1996) Retinal venous sheathing and the blood-retinal barrier in multiple sclerosis. Arch Ophthalmol 114:34–39
Rucker CW (1972) Sheathing of the retinal veins in multiple sclerosis; review of pertinent literature. Mayo Clin Proc 47:335–340
Prineas JW, Wright RG (1978) Macrophages, lymphocytes, and plasma cells in the perivascular compartment in chronic multiple sclerosis. Lab Investig 38:409–421
Towler HM, Lightman S (2000) Symptomatic intraocular inflammation in multiple sclerosis. Clin Exp Ophthalmol 28:97–102. doi:10.1046/j.1442-9071.2000.00270.x
Rothova A (2000) Ocular involvement in sarcoidosis. Br J Ophthalmol 84:110–116. doi:10.1136/bjo.84.1.110
Jones NP (2002) Sarcoidosis. Curr Opin Ophthalmol 13:393–396. doi:10.1097/00055735-200212000-00009
DeRosa AJ, Margo CE, Orlick ME (1995) Hemorrhagic retinopathy as the presenting manifestation of sarcoidosis. Retina 15:422–427
Ohara K, Okubo A, Saski H (1995) Branch retinal vein occlusion in a child with ocular sarcoidosis. Am J Ophthalmol 119:806–807
Kimmel AS, McCarthy MJ, Blodi CF, Folk JC (1989) Branch retinal vein occlusion in sarcoidosis. Am J Ophthalmol 107:561–562
Duker JS, Brown GC, McNamara JA (1988) Proliferative sarcoid retinopathy. Ophthalmology 95:1680–1686
Siam AL, Meegan JM, Gharbawi KF (1980) Rift Valley fever ocular manifestations: observations during the 1977 epidemic in Egypt. Br J Ophthalmol 64:366–374. doi:10.1136/bjo.64.5.366
Al-Hazmi A, Al-Rajhi AA, Abboud EB, Agaola EA, Al-Hazmi M, Saadi R, Ahmed N (2005) Ocular complications of Rift Valley fever outbreak in Saudi Arabia. Ophthalmology 112:313–318. doi:10.1016/j.ophtha.2004.09.018
Kaiser PK, Lee MS, Martin DA (2003) Occlusive vasculitis in a patient with concomitant West Nile virus infection. Am J Ophthalmol 136:928–930. doi:10.1016/S0002-9394(03)00866-3
Chan CK, Limstrom SA, Tarasewicz DG, Lin SG (2006) Ocular features of West Nile virus infection in North America: a study of 14 eyes. Ophthalmology 113:1539–1546. doi:10.1016/j.ophtha.2006.04.021
Teitelbaum BA, Newman TL, Tresley DJ (2007) Occlusive retinal vasculitis in a patient with West Nile virus. Clin Exp Optom 90:463–467. doi:10.1111/j.1444-0938.2007.00140.x
Herbort CP, Cimino L, Abu El-Asrar AM (2005) Ocular vasculitis: a multidisciplinary approach. Curr Opin Rheumatol 17:25–33. doi:10.1097/01.bor.0000149083.16639.7e
Metha S (2002) Role of computed chest tomography (CT scan) in tuberculous retinal vasculitis. Ocul Immunol Inflamm 10:151–155. doi:10.1076/ocii.10.2.151.13978
Lee SS, Liu YC, Chen YS, Tsai HC, Wann SR, Lin HH (2008) Comparison of interferon-gamma release assay and the tuberculin skin test for contact investigation of tuberculosis in BCG-vaccinated health care workers. Scand J Infect Dis 40:373–380. doi:10.1080/00365540701730743
Acknowledgements
The authors thank Ms. Connie B. Unisa-Marfil for secretarial work. This work was supported by Medical Research Chair in Ophthalmology funded by Dr. Naser Al-Rasheed.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Abu El-Asrar, A.M., Herbort, C.P. & Tabbara, K.F. A clinical approach to the diagnosis of retinal vasculitis. Int Ophthalmol 30, 149–173 (2010). https://doi.org/10.1007/s10792-009-9301-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10792-009-9301-3