Advertisement

The Clinical Characteristics of Noninfectious Occlusive Retinal Vasculitis

Published:April 23, 2021DOI:https://doi.org/10.1016/j.oret.2021.04.012

      Purpose

      To characterize the clinical features of occlusive retinal vasculitis (ORV).

      Design

      Retrospective case series.

      Participants

      Forty-two patients with ORV.

      Methods

      A retrospective chart review identified all patients with ORV seen at the University of Colorado uveitis service between January 2013 and April 2020. All included patients demonstrated noninfectious uveitis and evidence of vascular occlusion in the presence of retinal vascular inflammation on widefield fluorescein angiography.

      Main Outcome Measures

      Demographic data, visual acuity, clinical findings, and fluorescein angiography findings.

      Results

      We identified 73 eyes from 42 patients (15 men, 27 women) with ORV. Thirty-one of 42 patients had bilateral disease. Most eyes (54/73) showed mixed arteriolar and venous vasculitis compared with primarily arteriolar (6/73) or venous (15/73) vasculitis. Thirteen of 42 patients had an underlying systemic condition, most commonly granulomatosis with polyangiitis; however, bilaterality was not associated with a systemic condition. Retinal nonperfusion was present equally in zone 2 (28/73) and zone 3 (28/73) compared with zone 1 (16/73). Retinal or iris neovascularization was present in 25 of 73 eyes. Eighteen of 42 patients required more than 1 immunosuppressive medication (average, 1.33) to prevent progressive vascular occlusive disease.

      Conclusions

      Occlusive retinal vasculitis is a heterogeneous entity with significant risk of visual impairment. Systemic disease was more prevalent in this specific cohort compared with cohorts from prior studies of retinal vasculitis.

      Keywords

      Abbreviations and Acronyms:

      GPA (granulomatosis with polyangiitis), logMAR (logarithm of the minimum angle of resolution), ORV (occlusive retinal vasculitis), TREX1 (Three Prime Repair Exonuclease 1)
      To read this article in full you will need to make a payment
      Subscribe to Ophthalmology Retina
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Jabs D.A.
        • Nussenblatt R.B.
        • Rosenbaum J.T.
        Standardization of Uveitis Nomenclature Working Group. Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop.
        Am J Ophthalmol. 2005; 140: 509-516
        • Prete M.
        • Indiveri F.
        • Perosa F.
        Vasculitides: proposal for an integrated nomenclature.
        Autoimmun Rev. 2016; 15: 167-173
        • Datoo O’Keefe G.A.
        • Rao N.
        Retinal vasculitis: a framework and proposal for a classification system.
        Surv Ophthalmol. 2020; 66: 54-67
        • Cupps T.R.
        • Fauci A.S.
        The vasculitides.
        Major Probl Intern Med. 1981; 21: 1-211
        • Rosenbaum J.T.
        • Robertson Jr., J.E.
        • Watzke R.C.
        Retinal vasculitis—a primer.
        West J Med. 1991; 154: 182-185
        • Ali A.
        • Ku J.H.
        • Suhler E.B.
        • et al.
        The course of retinal vasculitis.
        Br J Ophthalmol. 2014; 98: 785-789
        • Rosenbaum J.T.
        • Sibley C.H.
        • Lin P.
        Retinal vasculitis.
        Curr Opin Rheumatol. 2016; 28: 228-235
        • Rosenbaum J.T.
        • Ku J.
        • Ali A.
        • et al.
        Patients with retinal vasculitis rarely suffer from systemic vasculitis.
        Semin Arthritis Rheum. 2012; 41: 859-865
        • Graham E.M.
        • Stanford M.R.
        • Sanders M.D.
        • et al.
        A point prevalence study of 150 patients with idiopathic retinal vasculitis: 1. Diagnostic value of ophthalmological features.
        Br J Ophthalmol. 1989; 73: 714-721
        • Ku J.H.
        • Ali A.
        • Suhler E.B.
        • et al.
        Characteristics and visual outcome of patients with retinal vasculitis.
        Arch Ophthalmol. 2012; 130: 1261-1266
        • Hupy M.L.
        • Pecen P.E.
        • Kolfenbach J.R.
        • et al.
        Purtscher’s-like retinopathy secondary to granulomatosis with polyangiitis following autologous stem cell transplantation.
        Retin Cases Brief Rep. 2020; (In press)https://doi.org/10.1097/ICB.0000000000001002
        • International Study Group for Behçet’s Disease
        Criteria for diagnosis of Behçet’s disease.
        Lancet. 1990; 335: 1078-1080
        • Holland G.N.
        • Buhles Jr., W.C.
        • Mastre B.
        • Kaplan H.J.
        A controlled retrospective study of ganciclovir treatment for cytomegalovirus retinopathy. Use of a standardized system for the assessment of disease outcome. UCLA CMV Retinopathy Study Group.
        Arch Ophthalmol. 1989; 107: 1759-1766
        • Aristodemou P.
        • Stanford M.
        Therapy insight: the recognition and treatment of retinal manifestations of systemic vasculitis.
        Nat Clin Pract Rheumatol. 2006; 2: 443-451
        • George R.K.
        • Walton R.C.
        • Whitcup S.M.
        • Nussenblatt R.B.
        Primary retinal vasculitis. Systemic associations and diagnostic evaluation.
        Ophthalmology. 1996; 103: 384-389
        • Saurabh K.
        • Das R.R.
        • Biswas J.
        • Kumar A.
        Profile of retinal vasculitis in a tertiary eye care center in Eastern India.
        Indian J Ophthalmol. 2011; 59: 297-301
        • Eales H.
        Cases of retinal hemorrhage associated with epistaxis and constipation.
        Birmingham Med Rev. 1880; 9: 262-273
        • Saxena S.
        • Kumar D.
        New classification system-based visual outcome in Eales’ disease.
        Indian J Ophthalmol. 2007; 55: 267-269