Skip to main content

Advertisement

SpringerLink
Log in

Ocular Vasculitis

  • VASCULITIS (LR ESPINOZA, SECTION EDITOR)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Vasculitis is the inflammation of blood vessels that leads to loss of perfusion and ischemia with necrosis. When this occurs in the orbit, the consequences are typically very conspicuous and can be devastating with decreased quality of life and loss of vision. Systemic vasculitides are often related to ophthalmic disorders, which can serve as the first diagnostic manifestation of potentially life-threatening disease. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (e.g., granulomatosis with polyangiitis), Behcet’s disease, rheumatoid arthritis, sarcoidosis, and systemic lupus erythematosus are a few of the diseases commonly associated with ocular vasculitis. Collaboration between ophthalmologists and rheumatologists is important in the successful diagnosis and treatment of patients with vasculitis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of Importance •• Of major importance

  1. Rothova A, Suttorp-van Schulten MSA, Treffers WF, et al. Causes and frequency of blindness in patients with intraocular inflammatory disease. Br J Ophthalmol. 1996;80:332–6.

    Article  PubMed  CAS  Google Scholar 

  2. •• Holle JU, Gross WL, Latza U, et al. Improved outcome in 445 patients with Wegener’s granulomatosis in a German vasculitis center over four decades. Arthritis Rheum. 2011;63:257–66. A comparative study showing improved outcomes with the advent of improved therapeutic procedures.

    Article  PubMed  Google Scholar 

  3. • Zlatanovic GD, Jovanovic SV, Zivkovic MLJ, et al. The efficacy of novel therapeutic modalities of isolated ocular vasculitis vs ocular vasculitis as a systemic disease. Med Glas Ljek Komore Zenicko-doboj Kantona. 2012;9:66–73. This study brings to light the overall morbidity still associated with vasculitic diseases.

    Google Scholar 

  4. Pagnini I, Zannin ME, Vittadello F, et al. Clinical features and outcome of Cogan syndrome. J Pediatr. 2012;160:303–7.

    Article  PubMed  Google Scholar 

  5. •• Schmidt J, Pulido JS, Matteson EL. Ocular manifestations of systemic disease: antineutrophil cytoplasmic antibody-associated vasculitis. Curr Opin Ophthalmol. 2011;22:489–95. A thorough and important review of ANCA-associated vasculitides.

    Article  PubMed  Google Scholar 

  6. Yu EN, Jurkunas U, Rubin PA, et al. Obliterative microangiopathy presenting as chronic conjunctivitis in a patient with relapsing polychondritis. Cornea. 2006;25:621–2.

    Article  PubMed  Google Scholar 

  7. Chung YM, Lin YC, Huang DF, et al. Conjunctival biopsy in sarcoidosis. J Chin Med Assoc. 2006;69:472–7.

    Article  PubMed  Google Scholar 

  8. Sainz de la Maza M, Molina N, Gonzalez-Gonzalez LA, et al. Clinical characteristics of a large cohort of patients with scleritis and Episcleritis. Ophthalmology. 2012;119:43–50.

    Article  PubMed  Google Scholar 

  9. Katz MS, Chuck RS, Gritz DC. Scleritis and episcleritis. Ophthalmology. 2012;119:1715.

    Article  PubMed  Google Scholar 

  10. Shepherd III JB. Ocular vasculitis. Curr Rheumatol Rep. 2003;5:100–6.

    Article  PubMed  Google Scholar 

  11. • Gregory 2nd AC, Kempen JH, Daniel E, et al. Risk factors for loss of visual acuity among patients with Uveitis associated with juvenile idiopathic arthritis: the systemic immunosuppressive Therapy for Eye Diseases Study. Ophthalmology. 2013;120:186–92. A study of 327 patients with 596 affected eyes that delves into the causes of vision loss.

    Article  PubMed  Google Scholar 

  12. Bonfioli AA, Damico FM, Curi AL, et al. Intermediate uveitis. Semin Ophthalmol. 2005;20:147–54.

    Article  PubMed  Google Scholar 

  13. Herbort CP, Cimino L, AbuElAsrar AM. Ocular vasculitis: a multidisciplinary approach. Curr Opin Rheumatol. 2005;17:25–33.

    Article  PubMed  Google Scholar 

  14. Herbort CP, Neri P, El Asrar AA, et al. Is ICGA still relevant in inflammatory eye disorders? Why this question has to be dealt with separately from other eye conditions. Retina. 2012;32:1701–3.

    Article  PubMed  Google Scholar 

  15. • Androudi S, Dastiridou A, Symeonidis C, et al. Retinal vasculitis in rheumatic disease: an unseen burden. Clin Rheumatol. 2013;32:7–13. A good review of retinal vasculitis with detailed ocular findings.

    Article  PubMed  Google Scholar 

  16. Espinoza G. Orbital inflammatory pseudotumors: etiology, differential diagnosis, and management. Curr Rheumatol Rep. 2010;12:443–7.

    Article  PubMed  Google Scholar 

  17. Harper SL, Letko E, Samson CM, et al. Wegener’s Granulomatosis: the relationship between ocular and systemic disease. J Rheumatol. 2001;28:1025–32.

    PubMed  CAS  Google Scholar 

  18. Bullen CL, Liesegang TJ, McDonald TJ, et al. Ocular complications of Wegener’s granulomatosis. Ophthalmology. 1983;90:279–90.

    PubMed  CAS  Google Scholar 

  19. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116:488–98.

    Article  PubMed  CAS  Google Scholar 

  20. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33:1101–7.

    Article  PubMed  CAS  Google Scholar 

  21. Tervaert JW, van der Woude FJ, Fauci AS, et al. Association between active Wegener’s granulomatosis and aticytoplasmic antibodies. Arch Intern Med. 1989;149:2461–5.

    Article  PubMed  CAS  Google Scholar 

  22. Nolle B, Specks U, Ludemann J, et al. Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis. Ann Intern Med. 1989;111:28–40.

    Article  PubMed  CAS  Google Scholar 

  23. Holle JU, Dubrau C, Herlyn K, et al. Rituximab for refractory granulomatosis with polyangiitis (Wegener’s granulomatosis): comparison of efficacy in granulomatous versus vasculitic manifestations. Ann Rheum Dis. 2012;71:327–33.

    Article  PubMed  CAS  Google Scholar 

  24. Wegener’s Granulomatosis Etanercept Trial Research Group. Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J Med. 2005;352:351–61.

    Article  Google Scholar 

  25. Fujita M, Igarachi T, Kurai T, et al. Correlation between dry eye and rheumatoid arthritis activity. Am J Ophthalmol. 2005;140:808–13.

    Article  PubMed  Google Scholar 

  26. Galor A, Thorne JE. Scleritis and peripheral ulcerative keratitis. Rheum Dis Clin North Am. 2007;33:835–54.

    Article  PubMed  Google Scholar 

  27. Foster CS, Forstot SL, Wilson LA. Mortality rate in rheumatoid arthritis patients developing necrotizing scleritis or peripheral ulcerative keratitis: effects of systemic immunosuppression. Ophthalmology. 1984;91:1253–63.

    PubMed  CAS  Google Scholar 

  28. Kapetanovic MC, Lindqvist E, Geborek P, et al. Long-term mortality rate in rheumatoid arthritis patients with disease onset in the 1980s. Scand J Rheumatol. 2011;40:433–8.

    Article  PubMed  CAS  Google Scholar 

  29. Ideguchi H, Suda A, Takeno M, et al. Behcet disease: evolution of clinical manifestations. Medicine. 2011;90:125–32.

    Article  PubMed  Google Scholar 

  30. Kotter I, Zierhut M, Eckstein AK, et al. Human recombinant interferon alfa-2a for the treatment of Behcet’s disease with sight threatening posterior or panuveitis. Br J Ophthalmol. 2003;87:423–31.

    Article  PubMed  CAS  Google Scholar 

  31. Oliveira AC, Buosi AL, Dutra LA, et al. Behcet disease: clinical features and management in a Brazilian tertiary hospital. J Clin Rheumatol. 2011;17:416–20.

    Article  PubMed  Google Scholar 

  32. • Markomichelakis N, Delicha E, Masselos S, et al. Intravitreal infliximab for sight-threatening relapsing uveitis in Behcet disease: a pilot study in 15 patients. Am J Ophthalmol. 2012;154:534–541.e1. This study shows a novel use of infliximab with good results in Behcet’s disease.

    Article  PubMed  CAS  Google Scholar 

  33. Suhler EB, Smith JR, Wertheim MS, et al. A prospective trial of infliximab therapy for refractory Uveitis. Arch Ophthalmol. 2005;123:903–12.

    Article  PubMed  CAS  Google Scholar 

  34. Sobrin L, Kim EC, Christen W, et al. Infliximab therapy for the treatment of refractory ocular inflammatory disease. Arch Ophthalmol. 2007;125:895–900.

    Article  PubMed  CAS  Google Scholar 

  35. Murphy CC, Ayliffe WH, Booth A, et al. Tumor necrosis factor alpha blockade with Infliximab for refractory Uveitis and scleritis. Ophthalmology. 2004;111:352–6.

    Article  PubMed  Google Scholar 

  36. Nichols JC, Ince A, Akduman L, et al. Interferon-alpha 2a treatment of Neuro-Behcet Disease. J Neuro-Ophthalmol. 2001;21:109–11.

    Article  CAS  Google Scholar 

  37. Jabs DA, Rosenbaum JT, Foster CS, et al. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol. 2000;130:492–513.

    Article  PubMed  CAS  Google Scholar 

  38. Rudwaleit M, Rodevand E, Holck P, et al. Adalimumab effectively reduces the rate of anterior uveitis flares in patients with active ankylosing spondylitis: results of a prospective open-label study. Ann Rheum Dis. 2009;68:696–701.

    Article  PubMed  CAS  Google Scholar 

  39. Tynjala P, Kotaniemi K, Lindahl P, et al. Adalimumab in juvenile idiopathic arthritis-associated chronic anterior uveitis. Rheumatology. 2008;47:339–44.

    Article  PubMed  CAS  Google Scholar 

  40. • Suhler EB, Lowder CY, Goldstein DA, et al. Adalimumab therapy for refractory uveitis: results of a multicenter, open-label, prospective trial. Br J Ophthalmol. 2013;97:481–6. Well designed prospective study on adalimumab by a group that also has studied infliximab. The study draws comparisons between the growing body of literature regarding these medicines in the treatment of uveitis.

    Article  PubMed  Google Scholar 

  41. Dobner BC, Max R, Becker MD, et al. A three-centre experience with adalimumab for the treatment of non-infectious uveitis. Br J Ophthalmol. 2013;97:134–8.

    Article  PubMed  Google Scholar 

  42. Androudi S, Tsironi E, Kalogeropoulos C, et al. Intravitreal Adalimumab for refractory uveitis-related macular edema. Ophthalmology. 2010;117:1612–6.

    Article  PubMed  Google Scholar 

  43. • Sellas A, Rodriguez B, Barcelo M, et al. Efficacy and safety of TNF antagonists in sarcoidosis: data from the Spanish registry of biologics BIOBADASER and a systematic review. Semin Arthritis Rheum. 2012;42:89–103. An extensive review of the use of TNF antagonists and their role in the treatment of sarcoidosis.

    Article  Google Scholar 

  44. Sandborn WJ, Hanauer SB, Katz S, et al. Etanercept for active Crohn’s disease: a randomized, double-blind, placebo-controlled trial. Gastroenterology. 2001;121:1088–94.

    Article  PubMed  CAS  Google Scholar 

  45. Silva F, Cisternas M, Specks U. TNF-alpha Blocker Therapy and Solid Malignancy Risk in ANCA-Associated Vasculitis. Curr Rheumatol Rep. 2012;14:501–8.

    Article  PubMed  CAS  Google Scholar 

  46. Arida A, Fragiadaki K, Giavri E, et al. Anti-TNF agents for Behcet’s disease: analysis of published data on 369 patients. Semin Arthritis Rheum. 2011;41:61–70.

    Article  PubMed  CAS  Google Scholar 

  47. • Pulido JS, Pulido JE, Michet CJ, et al. More questions than answers: a call for a moratorium on the use of intravitreal infliximab outside of a well-designed trial. Retina. 2010;30:1–5. A thorough discussion of intravitreal biologics and ethical dilemmas of treatment in refractory cases of disease.

    Article  PubMed  Google Scholar 

  48. Iaccheri B, Androudi S, Bocci EB, et al. Rituximab treatment for persistent scleritis associated with rheumatoid arthritis. Ocul Immunol Inflamm. 2010;18:223–5.

    Article  PubMed  CAS  Google Scholar 

  49. Chauhan S, Kamal A, Thompson RN, et al. Rituximab for treatment of scleritis associated with rheumatoid arthritis. Br J Ophthalmol. 2009;93:984–5.

    Article  PubMed  CAS  Google Scholar 

  50. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis. N Engl J Med. 2010;363:221–32.

    Article  PubMed  CAS  Google Scholar 

  51. Kitzmann AS, Pulido JS, Mohney BG, et al. Intraocular use of rituximab. Eye. 2007;21:1524–7.

    Article  PubMed  CAS  Google Scholar 

  52. Yeh S, Wilson DJ. Combination intravitreal rituximab and methotrexate for massive subretinal lymphoma. Eye. 2010;24:1625–7.

    Article  PubMed  CAS  Google Scholar 

Download references

Compliance with Ethics Guidelines

Conflict of Interest

Gabriela M. Espinoza, Ankit Desai, and Levent Akduman declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Authors and Affiliations

  1. Department of Ophthalmology, Saint Louis University School of Medicine, Saint Louis University Eye Institute, 1755 South Grand Blvd, St. Louis, MO, 63104, USA

    Gabriela M. Espinoza, Ankit Desai & Levent Akduman

Authors
  1. Gabriela M. Espinoza
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ankit Desai
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Levent Akduman
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Gabriela M. Espinoza.

Additional information

This article is part of the Topical Collection on Vasculitis

Rights and permissions

Reprints and permissions

About this article

Cite this article

Espinoza, G.M., Desai, A. & Akduman, L. Ocular Vasculitis. Curr Rheumatol Rep 15, 355 (2013). https://doi.org/10.1007/s11926-013-0355-x

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11926-013-0355-x

Keywords

Search

Navigation