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Published in: Clinical Rheumatology 1/2013

01-01-2013 | Review Article

Retinal vasculitis in rheumatic diseases: an unseen burden

Authors: Sofia Androudi, Anna Dastiridou, Chrysanthos Symeonidis, Leila Kump, Anna Praidou, Periklis Brazitikos, Shree K. Kurup

Published in: Clinical Rheumatology | Issue 1/2013

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Abstract

Retinal vascular inflammation, a potentially blinding condition (herein: retinal vasculitis (RV)) is commonly associated with a heterogeneous group of diseases characterized by systemic inflammatory cell infiltration and/or necrosis of blood vessel walls. RV may arise as an isolated ocular disorder, as part of systemic vasculitis (Wegener’s granulomatosis and Adamantiadis–Behcet Disease), or it can be secondary to an underlying connective tissue disease (systemic lupus erythematosus, sarcoidosis, and rheumatoid arthritis), systemic infection, or malignancy. Depending on the type of RV, it can be a potentially disabling condition, in the short or long term. Early diagnosis is the key to successful treatment and better prognosis. However, early diagnosis can be difficult, because these conditions usually present with nonspecific visual symptoms for a long period before diagnostic manifestations occur. The retina should be examined in warranted patients with verified rheumatic disease, since retinal vasculitis may be asymptomatic at the beginning (peripheral retinal disease). RV can be detected clinically (often accompanied by uveitis, scleritis, or macular edema) or revealed on fluorescein fundus angiography, even if minimal signs of retinal vessel inflammation are present. RV may also represent one of the possible extra-articular manifestations of the rheumatic disease. Rheumatologists should be familiar with the ocular manifestations of these disorders, since they may not only be sight-threatening, but more importantly, could be the presenting or even the very first manifestations of active, potentially lethal systemic disease in a patient with nonspecific rheumatologic presentation.
Literature
1.
go back to reference Gritz DC, Wong IG (2004) Incidence and prevalence of uveitis in Northern California; the Northern California Epidemiology of Uveitis Study. Ophthalmology 111:491–500PubMedCrossRef Gritz DC, Wong IG (2004) Incidence and prevalence of uveitis in Northern California; the Northern California Epidemiology of Uveitis Study. Ophthalmology 111:491–500PubMedCrossRef
2.
go back to reference Foster CS, Foster CS (1987) Ocular manifestations of the nonrheumatic acquired collagen vascular diseases. In: Smolin G, Thoft RA (eds) The cornea: scientific foundations and clinical practice, 2nd edn. Little, Brown, Boston, pp 352–357 Foster CS, Foster CS (1987) Ocular manifestations of the nonrheumatic acquired collagen vascular diseases. In: Smolin G, Thoft RA (eds) The cornea: scientific foundations and clinical practice, 2nd edn. Little, Brown, Boston, pp 352–357
3.
go back to reference Jabs DA, Fine SL, Hochberg MC et al (1986) Severe retinal vaso-occlusive disease in systemic lupus erythematosus. Arch Ophthalmol 104:588 Jabs DA, Fine SL, Hochberg MC et al (1986) Severe retinal vaso-occlusive disease in systemic lupus erythematosus. Arch Ophthalmol 104:588
4.
go back to reference Wong K, Ai E, Jones JV, Young D (1981) Visual loss as the initial symptom of systemic lupus erythematosus. Am J Ophthalmol 92:238PubMed Wong K, Ai E, Jones JV, Young D (1981) Visual loss as the initial symptom of systemic lupus erythematosus. Am J Ophthalmol 92:238PubMed
5.
go back to reference Stafford-Brady FJ, Urowitz MB, Gladman DD, Easterbrook M (1988) Lupus retinopathy: patterns, associations and prognosis. Arthritis Rheum 31:1105PubMedCrossRef Stafford-Brady FJ, Urowitz MB, Gladman DD, Easterbrook M (1988) Lupus retinopathy: patterns, associations and prognosis. Arthritis Rheum 31:1105PubMedCrossRef
6.
go back to reference Baehr G, Klemperer P, Schifrin A (1935) A diffuse disease of the peripheral circulation (usually associated with lupus erythematosus and endocarditis). Trans Assoc Am Physicians 50:139 Baehr G, Klemperer P, Schifrin A (1935) A diffuse disease of the peripheral circulation (usually associated with lupus erythematosus and endocarditis). Trans Assoc Am Physicians 50:139
7.
go back to reference Lanham JG, Barrie T, Kohner EM, Hughs RV (1982) SLE retinopathy: evaluation by fluorescein angiography. Ann Rheum Dis 41:473PubMedCrossRef Lanham JG, Barrie T, Kohner EM, Hughs RV (1982) SLE retinopathy: evaluation by fluorescein angiography. Ann Rheum Dis 41:473PubMedCrossRef
8.
go back to reference Klinkhoff AV, Beattie CW, Chalmers A (1986) Retinopathy in systemic lupus erythematosus. Relationship to disease activity. Arthritis Rheum 29:1152PubMedCrossRef Klinkhoff AV, Beattie CW, Chalmers A (1986) Retinopathy in systemic lupus erythematosus. Relationship to disease activity. Arthritis Rheum 29:1152PubMedCrossRef
9.
go back to reference Montehermoso A, Cervera R, Font J, Ramos-Casals M, García-carrasco M, Formiga F, Callejas JL, Jorfán M, Griñó MC, Ingelmo M (1999) Association of antiphospholipid antibodies with retinal vascular disease in systemic lupus erythematosus. Semin Arthritis Rheum 28(5):326–332PubMedCrossRef Montehermoso A, Cervera R, Font J, Ramos-Casals M, García-carrasco M, Formiga F, Callejas JL, Jorfán M, Griñó MC, Ingelmo M (1999) Association of antiphospholipid antibodies with retinal vascular disease in systemic lupus erythematosus. Semin Arthritis Rheum 28(5):326–332PubMedCrossRef
10.
go back to reference Dougal MA, Evans LS, McClellan KR, Robinson J (1983) Central retinal arterial occlusion in systemic lupus erythematosus. Ann Ophthalmol 15:38PubMed Dougal MA, Evans LS, McClellan KR, Robinson J (1983) Central retinal arterial occlusion in systemic lupus erythematosus. Ann Ophthalmol 15:38PubMed
11.
go back to reference Ellis CJ, Hamer DB, Hunt TW et al (1964) Medical investigation of retinal vascular occlusions. Br Med J 2:1093PubMedCrossRef Ellis CJ, Hamer DB, Hunt TW et al (1964) Medical investigation of retinal vascular occlusions. Br Med J 2:1093PubMedCrossRef
12.
go back to reference Harvey AM, Shulman LE, Tumulty PA et al (1954) Systemic lupus erythematosus: review of the literature and clinical analysis of 138 cases. Medicine 33:291PubMedCrossRef Harvey AM, Shulman LE, Tumulty PA et al (1954) Systemic lupus erythematosus: review of the literature and clinical analysis of 138 cases. Medicine 33:291PubMedCrossRef
13.
go back to reference Carpenter MT, O’Doyle JE, Enzenauer RV et al (1994) Chorioditis in systemic lupus erythematosus. Am J Ophthalmol 117:535–536PubMed Carpenter MT, O’Doyle JE, Enzenauer RV et al (1994) Chorioditis in systemic lupus erythematosus. Am J Ophthalmol 117:535–536PubMed
14.
go back to reference Santos R, Barojas E, Alarcon-Segovia D, Ibanez G (1975) Retinal microangiopathy in systemic lupus erythematosus. Am J Ophthalmol 80:249PubMed Santos R, Barojas E, Alarcon-Segovia D, Ibanez G (1975) Retinal microangiopathy in systemic lupus erythematosus. Am J Ophthalmol 80:249PubMed
15.
go back to reference Lavina AM, Agarwal A, Hunyor A, Gass JD (2002) Lupus choroidopathy and choroidal effusions. Retina 22(5):643–647PubMedCrossRef Lavina AM, Agarwal A, Hunyor A, Gass JD (2002) Lupus choroidopathy and choroidal effusions. Retina 22(5):643–647PubMedCrossRef
16.
go back to reference Aronson AJ, Ordoñez NG, Diddie KR, Ernest JT (1979) Immune-complex deposition in the eye in systemic lupus erythematosus. Arch Intern Med 139(11):1312–1313PubMedCrossRef Aronson AJ, Ordoñez NG, Diddie KR, Ernest JT (1979) Immune-complex deposition in the eye in systemic lupus erythematosus. Arch Intern Med 139(11):1312–1313PubMedCrossRef
17.
go back to reference Tan EM, Cohen AS, Fries JF et al (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271PubMedCrossRef Tan EM, Cohen AS, Fries JF et al (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271PubMedCrossRef
18.
go back to reference Maumenee AE (1940) Retinal lesions in lupus erythematosus. Am J Ophthalmol 23:971 Maumenee AE (1940) Retinal lesions in lupus erythematosus. Am J Ophthalmol 23:971
19.
go back to reference Graham EM, Spalton DJ, Barnard RO et al (1985) Cerebral and retinal vascular changes in systemic lupus erythematosus. Ophthalmology 92:444–448PubMed Graham EM, Spalton DJ, Barnard RO et al (1985) Cerebral and retinal vascular changes in systemic lupus erythematosus. Ophthalmology 92:444–448PubMed
20.
go back to reference Wikinson M, Torrance WN (1967) Clinical background of rheumatoid vascular disease. Ann Rheum Dis 26:475CrossRef Wikinson M, Torrance WN (1967) Clinical background of rheumatoid vascular disease. Ann Rheum Dis 26:475CrossRef
21.
go back to reference Scott DG, Bacon PA, Tribe CR (1981) Systemic rheumatoid vasculitis: a clinical and laboratory study of 50 cases. Medicine (Balitmore) 60:288–297 Scott DG, Bacon PA, Tribe CR (1981) Systemic rheumatoid vasculitis: a clinical and laboratory study of 50 cases. Medicine (Balitmore) 60:288–297
22.
go back to reference Foster CS, Forstot SL, Wilson LA (1984) Mortality rate in rheumatoid arthritis patients developing necrotizing scleritis or peripheral ulcerative keratitis. Effects of systemic immunosuppression. Ophthalmology 91:1253PubMed Foster CS, Forstot SL, Wilson LA (1984) Mortality rate in rheumatoid arthritis patients developing necrotizing scleritis or peripheral ulcerative keratitis. Effects of systemic immunosuppression. Ophthalmology 91:1253PubMed
23.
go back to reference Giordano N, D’Ettorre M, Biasi G, Fioravanti A, Moretti L, Marcolongo R (1990) Retinal vasculitis in rheumatoid arthritis: an angiographic study. Clin Exp Rheumatol 8(2):121–125PubMed Giordano N, D’Ettorre M, Biasi G, Fioravanti A, Moretti L, Marcolongo R (1990) Retinal vasculitis in rheumatoid arthritis: an angiographic study. Clin Exp Rheumatol 8(2):121–125PubMed
24.
go back to reference Matsuo T, Masuda I, Matsuo N (1998) Geographic choroiditis and retinal vasculitis in rheumatoid arthritis. Jpn J Ophthalmol 42:51–55PubMedCrossRef Matsuo T, Masuda I, Matsuo N (1998) Geographic choroiditis and retinal vasculitis in rheumatoid arthritis. Jpn J Ophthalmol 42:51–55PubMedCrossRef
25.
go back to reference Graham E, Stanford MR, Sanders MD et al (1989) A point prevalence study of 150 patients with retinal vasculitis: 1. Diagnostic value of ophthalmologic features. Br J Ophthalmol 73:714–721PubMedCrossRef Graham E, Stanford MR, Sanders MD et al (1989) A point prevalence study of 150 patients with retinal vasculitis: 1. Diagnostic value of ophthalmologic features. Br J Ophthalmol 73:714–721PubMedCrossRef
26.
go back to reference Smith JA, Foster CS (1996) Sarcoidosis and its ocular manifestations. Int Ophthalmol Clin 36:109–125PubMedCrossRef Smith JA, Foster CS (1996) Sarcoidosis and its ocular manifestations. Int Ophthalmol Clin 36:109–125PubMedCrossRef
27.
go back to reference Spalton D, Sanders M (1981) Fundus changes in histologically confirmed sarcoidosis. Br J Ophthalmol 65:348–358PubMedCrossRef Spalton D, Sanders M (1981) Fundus changes in histologically confirmed sarcoidosis. Br J Ophthalmol 65:348–358PubMedCrossRef
28.
go back to reference Bisighini S, Pagluiso L (1997) Retinal vasculitis: a diagnostic dilemma. Clin Eye Vis Care 9:71–84CrossRef Bisighini S, Pagluiso L (1997) Retinal vasculitis: a diagnostic dilemma. Clin Eye Vis Care 9:71–84CrossRef
29.
go back to reference Evereklioglu C (2005) Current concepts in the etiology and treatment of Behçet disease. Surv Ophthalmol 50:297–350PubMedCrossRef Evereklioglu C (2005) Current concepts in the etiology and treatment of Behçet disease. Surv Ophthalmol 50:297–350PubMedCrossRef
30.
go back to reference Kötter I, Zierhut M, Eckstein AK et al (2003) Human recombinant interferon alfa-2a for the treatment of Behçet’s disease with sight threatening posterior or panuveitis. Br J Ophthalmol 87:423–431PubMedCrossRef Kötter I, Zierhut M, Eckstein AK et al (2003) Human recombinant interferon alfa-2a for the treatment of Behçet’s disease with sight threatening posterior or panuveitis. Br J Ophthalmol 87:423–431PubMedCrossRef
31.
go back to reference Jabs DA, Rosenbaum JT, Fosters CS et al (2000) Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol 130:492–513PubMedCrossRef Jabs DA, Rosenbaum JT, Fosters CS et al (2000) Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol 130:492–513PubMedCrossRef
32.
go back to reference Tabbara KF, Al-Hemidan AI (2008) Infliximab effects compared to conventional therapy in the management of retinal vasculitis in Behçet disease. Am J Ophthalmol 146(6):845–50.e1PubMedCrossRef Tabbara KF, Al-Hemidan AI (2008) Infliximab effects compared to conventional therapy in the management of retinal vasculitis in Behçet disease. Am J Ophthalmol 146(6):845–50.e1PubMedCrossRef
33.
go back to reference Deuter CM, Kötter I, Wallace GR et al (2008 Jan) Behçet’s disease: ocular effects and treatment. Prog Retin Eye Res 27(1):111–136PubMedCrossRef Deuter CM, Kötter I, Wallace GR et al (2008 Jan) Behçet’s disease: ocular effects and treatment. Prog Retin Eye Res 27(1):111–136PubMedCrossRef
34.
go back to reference Bullen CL, Liesegang TJ, McDonald TJ et al (1983) Ocular complications of Wegener’s granulomatosis. Ophthalmology 90:279–290PubMed Bullen CL, Liesegang TJ, McDonald TJ et al (1983) Ocular complications of Wegener’s granulomatosis. Ophthalmology 90:279–290PubMed
35.
go back to reference Hoffman GS, Kerr GS, Leavitt RY et al (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116:488–498PubMed Hoffman GS, Kerr GS, Leavitt RY et al (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116:488–498PubMed
36.
go back to reference Kaufman AH, Niles JL, Foster CS (1994) ANCA test in ophthalmic inflammatory disease. Int Ophthalmol Clin 34:215–227PubMedCrossRef Kaufman AH, Niles JL, Foster CS (1994) ANCA test in ophthalmic inflammatory disease. Int Ophthalmol Clin 34:215–227PubMedCrossRef
37.
go back to reference Power WJ, Rodriguez A, Neves RA et al (1995) Disease relapse in patients with ocular manifestations of Wegener granulomatosis. Ophthalmology 102:154–160PubMed Power WJ, Rodriguez A, Neves RA et al (1995) Disease relapse in patients with ocular manifestations of Wegener granulomatosis. Ophthalmology 102:154–160PubMed
38.
go back to reference Haynes BF, Fishman ML, Fauci AS, Wolff SM (1977) The ocular manifestations of Wegener’s granlomatosis. Fifteen years experience and review of the literature. Am J Med 63:131–141PubMedCrossRef Haynes BF, Fishman ML, Fauci AS, Wolff SM (1977) The ocular manifestations of Wegener’s granlomatosis. Fifteen years experience and review of the literature. Am J Med 63:131–141PubMedCrossRef
39.
go back to reference Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA (1983) Ocular complications of WG. Ophthalmology 90:279–290PubMed Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA (1983) Ocular complications of WG. Ophthalmology 90:279–290PubMed
40.
go back to reference Pinching AJ, Lockwood CM, Pussell BA et al (1983) Wegener’s granulomatosis: observations on 18 patients with severe renal disease. Q J Med 208:435–460 Pinching AJ, Lockwood CM, Pussell BA et al (1983) Wegener’s granulomatosis: observations on 18 patients with severe renal disease. Q J Med 208:435–460
41.
go back to reference Cassan SM, Coles DT, Harrison E (1970) The concept of limited forms of Wegener’s granulomatosis. Am J Med 49:366PubMedCrossRef Cassan SM, Coles DT, Harrison E (1970) The concept of limited forms of Wegener’s granulomatosis. Am J Med 49:366PubMedCrossRef
42.
go back to reference Spalton DJ, Graham EM, Page NGR, Sanders MD (1981) Ocular changes in limited forms of Wegener’s granulomatosis. Br J Ophthalmol 65:553–563PubMedCrossRef Spalton DJ, Graham EM, Page NGR, Sanders MD (1981) Ocular changes in limited forms of Wegener’s granulomatosis. Br J Ophthalmol 65:553–563PubMedCrossRef
43.
go back to reference Dodds EM, Lowder CY, Meisler DM (1999) Posterior segment inflammation in HLA-B27+ acute anterior uveitis: clinical characteristics. Ocul Immunol Inflamm 7:85–92PubMedCrossRef Dodds EM, Lowder CY, Meisler DM (1999) Posterior segment inflammation in HLA-B27+ acute anterior uveitis: clinical characteristics. Ocul Immunol Inflamm 7:85–92PubMedCrossRef
44.
go back to reference Power WJ, Rodriguez A, Pedroza-Seres M et al (1998) Outcomes in anterior uveitis associated with the HLA-B27 haplotype. Ophthalmology 105:1646–1651PubMedCrossRef Power WJ, Rodriguez A, Pedroza-Seres M et al (1998) Outcomes in anterior uveitis associated with the HLA-B27 haplotype. Ophthalmology 105:1646–1651PubMedCrossRef
45.
go back to reference Rodriguez A, Akova YA, Pedroza-Seres M, Foster CS (1994) Posterior segment ocular manifestations in patients with HLA-B27-associated uveitis. Ophthalmology 101:1267–1274PubMed Rodriguez A, Akova YA, Pedroza-Seres M, Foster CS (1994) Posterior segment ocular manifestations in patients with HLA-B27-associated uveitis. Ophthalmology 101:1267–1274PubMed
46.
go back to reference Watts RA, Scott DG, Pusey CD, Lockwood CM (2000) Vasculitis-aims of therapy. An overview. Rheumatology 39:229–237PubMedCrossRef Watts RA, Scott DG, Pusey CD, Lockwood CM (2000) Vasculitis-aims of therapy. An overview. Rheumatology 39:229–237PubMedCrossRef
47.
go back to reference Hemady R, Tauber J, Foster CS (1991) Immunosuppressive drugs in immune and inflammatory ocular disease. Surv Ophthalmol 35:369–385PubMedCrossRef Hemady R, Tauber J, Foster CS (1991) Immunosuppressive drugs in immune and inflammatory ocular disease. Surv Ophthalmol 35:369–385PubMedCrossRef
48.
go back to reference Perez VL, Chavala SH, Ahmed M, Chu D et al (2004) Ocular manifestations and concepts of systemic vasculitides. Surv Ophthalmol 49:399–418PubMedCrossRef Perez VL, Chavala SH, Ahmed M, Chu D et al (2004) Ocular manifestations and concepts of systemic vasculitides. Surv Ophthalmol 49:399–418PubMedCrossRef
49.
go back to reference Kurup S (2008) Noncorticosteroid immune therapy for ocular inflammation. In: Tasman W, Jaeger EA (eds) Duane’s ophthalmology, Chptr 31. Lippincott Williams & Wilkins Kurup S (2008) Noncorticosteroid immune therapy for ocular inflammation. In: Tasman W, Jaeger EA (eds) Duane’s ophthalmology, Chptr 31. Lippincott Williams & Wilkins
50.
go back to reference Kurup S, Lew J, Byrnes G et al (2009) Therapeutic efficacy of intravitreal bevacizumab on posterior uveitis complicated by neovascularization. Acta Ophthalmol 87:349–352PubMedCrossRef Kurup S, Lew J, Byrnes G et al (2009) Therapeutic efficacy of intravitreal bevacizumab on posterior uveitis complicated by neovascularization. Acta Ophthalmol 87:349–352PubMedCrossRef
Metadata
Title
Retinal vasculitis in rheumatic diseases: an unseen burden
Authors
Sofia Androudi
Anna Dastiridou
Chrysanthos Symeonidis
Leila Kump
Anna Praidou
Periklis Brazitikos
Shree K. Kurup
Publication date
01-01-2013
Publisher
Springer-Verlag
Published in
Clinical Rheumatology / Issue 1/2013
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-012-2078-1

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