Uveitis pp 1-16 | Cite as

Noninfectious Anterior Uveitis

  • Kristin BiggeeEmail author
Part of the Current Practices in Ophthalmology book series (CUPROP)


Noninfectious anterior uveitis is the most common type of uveitis. It presents with a variable clinical course that is most commonly idiopathic. It can be associated with a heterogenous group of systemic and ocular disorders. HLA B27 positivity is the most common association and occurs in the majority of acute unilateral cases. The pathogenesis of noninfectious anterior uveitis is still poorly understood, but it is likely multifactorial, as multiple genetic and other alterations in immune response have been observed among different subtypes. Complications such as cataract, band keratopathy, glaucoma, macular edema, and hypotony can lead to visual impairment. The mainstay of treatment includes topical or local steroids, but steroid-sparing therapy should be considered in recalcitrant or severe cases.


Noninfectious anterior uveitis HLA B27 uveitis Differential diagnosis of anterior uveitis Treatment of anterior uveitis 


  1. 1.
    Jabs DA, Nussenblatt RB, Rosenbaum JT. Standardization of uveitis nomenclature (SUN) working group. Standardization of uveitis nomenclature for reporting clinical data. Results of the first international workshop. Am J Ophthalmol. 2005;140(3):509–16.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Lin P, Loh AR, Margolis TP, Acharya NR. Cigarette smoking as a risk factor of uveitis. Ophthalmology. 2010;117(3):585–90.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    McCannel CA, Holland GN, Helm CJ, et al. Causes of uveitis in the general practice of ophthalmology: UCLA Community-based uveitis study group. Am J Ophthalmol. 1996;121:35–46.CrossRefGoogle Scholar
  4. 4.
    Darrell RW, Wagener HP, Kurland LT. Epidemiology of uveitis. Incidence and prevalence in a small urban community. Arch Ophthalmol. 1962;68:502–14.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Gritz DC, Wong IG. Incidence and prevalence of uveitis in northern California. Ophthalmology. 2004;140:509–16.Google Scholar
  6. 6.
    Suhler EB, Llyod MJ, Dongseok C, et al. Incidence and prevalence of uveitis in veterans affairs medical centers of the Pacific northwest. Am J Ophthalmol. 2008;146(6):890–6.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Acharya NR, Tham VM, Esterberg E, et al. Incidence and prevalence of uveitis. Results from the Pacific ocular inflammation study. JAMA Ophthal. 2013;131(11):1405–12.CrossRefGoogle Scholar
  8. 8.
    Thorne JE, Suhler E, Skup M, et al. Prevalence of noninfectious uveitis in the United States a claims-based analysis. JAMA Ophthalmol. 2016;134(11):1237–45.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Sen NH, Davis J, Ucar D, et al. Gender disparities in ocular inflammatory disorders. Curr Eye Res. 2015;40(2):146–61.CrossRefGoogle Scholar
  10. 10.
    Bowling B. Kanski’s clinical ophthalmology: a systematic approach: Elsevier Health Sciences; London: 2015:. p398. E-BookGoogle Scholar
  11. 11.
    Ocampo VV, Foster CS, Baltatzis S. Surgical excision of iris nodules in the management of sarcoid uveitis. Ophthalmology. 2001;108(7):1296–9.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Jabs DA, Johns CJ. Ocular involvement in chronic sarcoidosis. Am J Ophthalmol. 1986;102:297–301.CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Rothova A, La Hey E, Baarsma GS, et al. Iris nodules in Fuchs’ heterochromic uveitis. Am J Ophthalmol. 1994;118:338–42.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Ebenezer D, Pistilli M, Kothari S, et al. Risk of ocular hypertension in adults with non-infectious uveitis. Ophthalmology. 2017;124(8):1196–208.CrossRefGoogle Scholar
  15. 15.
    Smith WM, Reddy MG, Hutcheson KA, et al. Rifabutin-associated hypopyon uveitis and retinal vasculitis with a history of acute myeloid leukemia. J Ophthalmic Inflamm Infect. 2012;2:149–52.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Nascimento H, Yasuta MK, Marquezan MC, et al. Uveitic band keratopathy: child and adult. J Ophthalmil Inflamm Infect. 2015;5:35.CrossRefGoogle Scholar
  17. 17.
    Khochtali S, Zina S, Kechida M, et al. Anterior uveitis as the presenting sign of chronic lymphocytic leukemia. J Fr Ophthalmol. 2017;40(9):e337–8.CrossRefGoogle Scholar
  18. 18.
    Chocron IM, Morrison DG, Friedman DL, et al. Ophthalmic manifestations of relapsing acute childhood leukemia. J AAPOS. 2015;19(3):284–6.CrossRefGoogle Scholar
  19. 19.
    Huang S, Suhrland MJ, Gritz D. Recurrent immunodeficiency-associated Burkitt lymphoma presenting as severe anterior uveitis. Ocul Oncol Pathol. 2015;2(2):62–5.CrossRefPubMedPubMedCentralGoogle Scholar
  20. 20.
    Kitao M, Hashida N, Nishida K. Recurrent pseudohypopyon in association with primary vitreoretinal lymphoma: a case report. BMC Ophthalmol. 2016;16:103.CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Pathanapitoon K, Dodds EM, Cunningham ET Jr, Rothova A. Clinical spectrum of HLA-B27-associated ocular inflammation. Ocul Immunol Inflamm. 2017;25(4):569–76.CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Zaidi AA, Ying GS, Daniel E. Systemic immunosuppressive therapy for eye diseases cohort study. Hypopyon in patients with uveitis. Ophthalmology. 2010;117(2):366–72.CrossRefGoogle Scholar
  23. 23.
    Tyagi M, Govindhari V, Pappuru RR, Ambiya V. Bilateral hypopyon uveitis in chronic myeloid leukemia. Ocul Oncol Pathol. 2017;4(1):12–5.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Birnbaum AD, Tessler HH, Goldstein DA. A case of hypopyon uveitis nonresponsive to steroid therapy and a review of anterior segment masquerade syndromes in childhood. J Pediatr Ophthalmol Strabismus. 2005;42(6):373–7.Google Scholar
  25. 25.
    Moorthy RS, Valluri S, Jampol LM. Drug-induced uveitis. Surv Ophthalmol. 1998;42(6):557–70.CrossRefGoogle Scholar
  26. 26.
    Acharya NR, Browne EN, Rao N, Mochizuki M. Distinguishing features of ocular sarcoidosis in an international cohort of uveitis patients. Ophthalmology. 2018;125(1):119–26.CrossRefGoogle Scholar
  27. 27.
    Quentin CD, Reiber H. Fuchs heterochromic cyclitis: rubella virus antibodies and genomes in aqueous humor. Am J Ophthalmol. 2004;138(1):46–54.CrossRefGoogle Scholar
  28. 28.
    Birnbaum AD, Tessler HH, Schultz KL, et al. Epidemiologic relationship between Fuchs heterochromic iridocyclitis and the United States rubella vaccination programs. Am J Ophthalmol. 2007;144(3):424–8.CrossRefGoogle Scholar
  29. 29.
    Takusagawa HL, Liu Y, Wiggs J. Infectious theories of Posner-Schlossman syndrome. Int Ophthalmol Clin. 2011;51(4):105–15.CrossRefGoogle Scholar
  30. 30.
    Rosenbaum JT. New developments in uveitis associated with HLA B27. Curr Opin Rheumatol. 2017;29:298–303.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Brewerton DA, Caffrey M, Nicholls A, et al. Acute anterior uveitis and HL-A 27. Lancet. 1973;302:994–6.CrossRefGoogle Scholar
  32. 32.
    Rudwaleit M, van der Heijde D, Landewe R, et al. The development of assessment of SpondyloArthritis international society classification criteria for axial spondyloarthritis (part II): validation and final selection. Ann Rheum Dis. 2009;68(6):777–83.CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Tay-Kearney ML, Schwam BL, Lowder C, et al. Clinical features and associated systemic diseases of HLA-B27 uveitis. Am J Ophthalmol. 1996;121(1):47–56.CrossRefGoogle Scholar
  34. 34.
    Stolwijk C, Boonen A, van Tubergen A, Reveille JD. Epidemiology of spondyloarthritis. Rheum Dis Clin N Am. 2012;38(3):441–76.CrossRefGoogle Scholar
  35. 35.
    Ozkan Y. Cardiac involvement in ankylosing spondylitis. J Clin Med Res. 2016;8(6):427–30.CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Kelly C, Iqbal K, Iman-Gutierriez L, et al. Lung involvement in inflammatory rheumatic diseases. Best Pract Res Clin Rheumatol. 2016;30(5):870–88.CrossRefGoogle Scholar
  37. 37.
    Schmitt SK. Reactive arthritis. Infect Dis Clin N Am. 2017;31(2):265–77.CrossRefGoogle Scholar
  38. 38.
    Rosenbaum JT. Uveitis in spondyloarthritis including psoriatic arthritis, ankylosing spondylitis, and inflammatory bowel disease. Clin Rheumatol. 2015;34(6):999–1002.CrossRefGoogle Scholar
  39. 39.
    Moradi A, Rowayda MA, Thorne JE. The role of gender in juvenile idiopathic arthritis-associated uveitis. J Ophthalmol. 2014;2014:461078.PubMedPubMedCentralGoogle Scholar
  40. 40.
    Coughlin L. Ophthalmic examinations in children with juvenile rheumatoid arthritis. Am Fam Physician. 2007;75(12):1886.Google Scholar
  41. 41.
    Sessa A, Meroni M, Battini G, et al. Acute renal failure due to idiopathic tubule-intestinal nephritis and uveitis: “TINU syndrome”. Case report and review of the literature. J Nephrol. 2000;13(5):377.PubMedGoogle Scholar
  42. 42.
    Pakzad-Vaezi K, Pepple KL. Tubulointerstitial nephritis and uveitis. Curr Opin Ophthalmol. 2017;28(6):629–35.CrossRefPubMedPubMedCentralGoogle Scholar
  43. 43.
    Zimmerman LE. Lens induced inflammation in human eyes. In: Maumenee AE, Silverstein AM, editors. Immunopathology of uveitis. Philadelphia, PA: Lippincott Williams & Wilkins; 1964. p. 221–32.Google Scholar
  44. 44.
    Rosenbaum JT, Samples JR, Seymour B, et al. Chemotactic activity of lens proteins and the pathogenesis of phacolytic glaucoma. Arch Ophthalmol. 1987;105(11):1582–4.CrossRefGoogle Scholar
  45. 45.
    Zemba M, Camburu G. Uveitis-Glaucoma-Hyphaema syndrome. General review. Rom J Ophthalmol. 2017;61(1):11–7.CrossRefPubMedPubMedCentralGoogle Scholar
  46. 46.
    Badakere SV, Senthil S, Turaga K, Garg P. Uveitis-Glaucoma-Hyphaema syndrome with in-the-bag placement of intraocular lens. BMJ Case Rep. 2016;2016:bcr2015213745.CrossRefPubMedPubMedCentralGoogle Scholar
  47. 47.
    Teo L, Chee SP. Retained lens fragment in the anterior segment as a cause of recurrent anterior uveitis. Int Ophthalmol. 2010;30(1):89–91.CrossRefGoogle Scholar
  48. 48.
    Hui JI, Fishler J, Karp CL, et al. Retained nuclear fragments in the anterior chamber after phacoemulsification with an intact posterior capsule. Ophthalmology. 2006;113(11):1949–53.CrossRefGoogle Scholar
  49. 49.
    Saraiya NV, Goldstein DA. Uveitis: drug induced uveitis. Practicing ophthalmologists learning system, 2017–2019. Am Acad Ophthalmol. 2017;Google Scholar
  50. 50.
    London NJ, Garg SJ, Moorthy RS, Cunningham ET. Drug-induced uveitis. J Ophthalmic Inflamm Infect. 2013;3(1):43.CrossRefPubMedPubMedCentralGoogle Scholar
  51. 51.
    Taylor SC, Hrisomalos F, Linette GP, Rao PK. A case of recurrent bilateral uveitis independently associated with dabrafenib and pembrolizumab therapy. Am J Ophthalmol Case Rep. 2016;2:23–5.CrossRefPubMedPubMedCentralGoogle Scholar
  52. 52.
    Sarny S, Neumayer M, Kofler J, El-Shabrawi Y. Ocular toxicity due to trametinib and dabrafenib. BMC Ophthalmol. 2017;17(1):146.CrossRefPubMedPubMedCentralGoogle Scholar
  53. 53.
    Karlin J, Gentzler R, Golen J. Bilateral anterior uveitis associated with nivolumab therapy. Ocul Immunol Inflamm. 2018;26(2):282–5.CrossRefGoogle Scholar
  54. 54.
    Numata S, Iwata Y, Okumura R, et al. Bilateral anterior uveitis and unilateral facial palsy due to ipilimumab for metastatic melanoma in an individual with human leukocyte antigen DR4: a case report. J Dermatol. 2018;45(1):113–4.CrossRefGoogle Scholar
  55. 55.
    Evans DM, Spencer CC, Pointon JJ, et al. Interaction between ERAP1 and HLA-B27 in ankylosing spondylitis implicates peptide handling in the mechanism for HLA-B27 in disease susceptibility. Nat Genet. 2011;43(8):761–7.CrossRefPubMedPubMedCentralGoogle Scholar
  56. 56.
    Cancino-Diaz JC, Vargas-Rodriguez L, Grinberg-Zylberbaum N, et al. High levels of IgG class antibodies to recombinant HSP60 kDa of Yersinia enterocolitica in sera of patients with uveitis. Br J Ophthalmol. 2004;88(2):247–50.CrossRefPubMedPubMedCentralGoogle Scholar
  57. 57.
    Garg AX, Pope JE, Thiessen-Philbrook H, et al. Arthritis risk after acute bacterial gastroenteritis. Rheumatology. 2008;47(2):200–4.CrossRefPubMedPubMedCentralGoogle Scholar
  58. 58.
    Lin P, Bach M, Asquith M, et al. HLA-B27 and human beta2-microglobulin affect the gut microbiota of transgenic rats. PLoS One. 2014;9:e105684.CrossRefPubMedPubMedCentralGoogle Scholar
  59. 59.
    Rosenbaum JT, Lin P, Asquith M. Does the microbiome cause B27-related acute anterior uveitis? Ocul Immunol Inflamm. 2016;24(4):440–4.CrossRefPubMedPubMedCentralGoogle Scholar
  60. 60.
    Chan AS, Sharma OP, Rao NA. Review for disease of the year: immunopathogenesis of ocular sarcoidosis. Ocul Immunol Inflamm. 2010;18(3):143–51.CrossRefGoogle Scholar
  61. 61.
    Emmi L, Brugnolo F, Salvati G, et al. Immunopathological aspects of Behcet’s disease. Clin Exp Rheumatol. 1995;13(6):687–91.PubMedGoogle Scholar
  62. 62.
    Sugi-Ikai N, Nakazawa M, Nakamura S, et al. Increased frequencies of interleukin-2 and interferon-gamma producing T cells in patients with active Behcet’s disease. Invest Ophthalmol Vis Sci. 1998;39(6):996–1004.PubMedGoogle Scholar
  63. 63.
    Hinks A, Ke X, Barton A, et al. Association of the IL2RA/CD25 gene with juvenile idiopathic arthritis. Arthritis Rheum. 2009;60(1):251–7.CrossRefPubMedPubMedCentralGoogle Scholar
  64. 64.
    Yanagimachi M, Miyamae T, Naruto T, et al. Association of HLA-A(∗)02:06 and HLA-DRB1(∗)04:05 with clinical subtypes of juvenile idiopathic arthritis. J Hum Genet. 2011;56(3):196–9.CrossRefGoogle Scholar
  65. 65.
    Awan MA, Agarwal PK, Watson DG, et al. Penetration of topical and sub-conjunctival corticosteroids into human aqueous humor and its therapeutic significance. Br J Ophthalmol. 2009;93(6):708–13.CrossRefGoogle Scholar
  66. 66.
    Sheppard JD, Foster S, Toyos MM, et al. Difluprednate 0.05% versus prednisolone acetate 1% for endogenous anterior uveitis: pooled efficacy analysis of two phase 3 studies. Ocul Immunol Inflamm. 2017;27:1–13.Google Scholar
  67. 67.
    Dick AD, Rosenbaum JT, Al-Dhibi HA, Fundamentals of Care for Uveitis International Consensus Group. Guidance on noncorticosteroid systemic immunomodulatory therapy in noninfectious uveitis: fundamentals of care for uveitis (FOCUS) initiative. Ophthalmology. 2018;125:757–73. pii:S0161-6420(17)32446-6. Epub ahead of printCrossRefGoogle Scholar
  68. 68.
    Benitez-Del-Castillo JM, Sanchez-Garcia J, Iradier T, Banares A. Sulfasalazine in the prevention of anterior uveitis associated with ankylosing spondylitis. Eye (Lond). 2000;14(Pt3A):340–3.CrossRefGoogle Scholar
  69. 69.
    Munoz-Fernandez S, Hidalgo V, Fernandez-Melon J, et al. Sulfasalazine reduces the number of flares of acute anterior uveitis over a one-year period. J Rheumatol. 2003;30(6):1277–9.PubMedGoogle Scholar
  70. 70.
    Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behcet’s syndrome. N Engl J Med. 1990;322(5):281–5.CrossRefPubMedPubMedCentralGoogle Scholar
  71. 71.
    Atienza-Mateo B, Calvo-Rio V, Beltran E, et al. Anti-interleukin 6 receptor tocilizumab in refractory uveitis associated with Behcet’s disease: multicenter retrospective study. Rheumatology. 2018; Epub ahead of print
  72. 72.
    Calvo-Rio V, Santos-Gomez M, Calvo I, et al. Anti-interleukin-6 receptor tocilizumab for severe juvenile idiopathic arthritis-associated uveitis refractory to anti-tumor necrosis factor therapy: a multicenter study of twenty five patients. Arthritis Rheumatol. 2017;69(3):668–75.CrossRefGoogle Scholar

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© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.Kaiser Northwest PermanentePortlandUSA

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