Article thumbnail

Update on peripheral ulcerative keratitis

By Ayse Yagci


Ulcerative inflammation of the cornea occurs in the perilimbal cornea, and is associated with autoimmune collagen vascular and arthritic diseases. Rheumatoid arthritis is the most frequent underlying disease. The tendency for peripheral location is due to the distinct morphologic and immunologic characteristics of the limbal conjunctiva, which provides access for circulating immune complexes to the peripheral cornea via the capillary network. Deposition of immune complexes in the terminal ends of limbal vessels initiates immune-mediated vasculitis, and causes inflammatory cell and protein leakage due to vessel wall damage. Development of peripheral ulcerative keratitis associated with systemic disease may represent worsening of a potentially life-threatening disease. Accompanying scleritis, particularly the necrotizing form, is usually observed in severe cases, which may result in corneal perforation and loss of vision. Although first-line treatment with systemic corticosteroids is indicated for acute phases, immunosuppressive and cytotoxic agents are required for treatment of peripheral ulcerative keratitis associated with multisystem disorders. Recently, infliximab, a chimeric antibody against proinflammatory cytokine tumor necrosis factor-alpha, was reported to be effective in cases refractory to conventional immunomodulatory therapy. The potential side effects of these therapies require close follow-up and regular laboratory surveillance

Topics: Review
Publisher: Dove Medical Press
OAI identifier:
Provided by: PubMed Central

Suggested articles


  1. (1990). An analysis of therapeutic decision making regarding immunosuppressive chemotherapy for peripheral ulcerative keratitis.
  2. Anti-TNF antibody therapy in rheumatoid arthritis and the risk of serious infections and malignancies. Systematic review and meta-analysis of rare harmful effects in randomized controlled trials.
  3. Antiinflammatory effects of amniotic membrane transplantation in ocular surface disorders.
  4. Autoimmune phenomenon in Mooren’s ulcer.
  5. Autoimmunity to a cornea associated stromal antigen in patients with Mooren’s ulcer.
  6. Clinical characteristics of a large cohort of patients with scleritis and episcleritis.
  7. Clinical characteristics of Mooren’s ulcer in South India.
  8. Comparison of antimetabolite drugs as corticosteroid-sparing therapy for noninfectious ocular inflammation.
  9. (2011). Corneal disease in rheumatoid arthritis.
  10. (1992). Cyclosporin A for the treatment of necrotising scleritis and corneal melting in patients with rheumatoid arthritis.
  11. (1995). Destructive corneal and scleral disease associated with rheumatoid arthritis: medical and surgical management.
  12. Diagnostic value of anti-neutrophil cytoplasmic antibodies in scleritis associated with Wegener’s granulomatosis.
  13. (2001). Differential efficacy of tumor necrosis factor inhibition in the management of inflammatory eye disease and associated rheumatic disease. Arthritis Care Res.
  14. (1981). Distribution of hemolytic complement activity in normal human donor cornea. Arch Ophthalmol.
  15. double-blind, placebocontrolled, pilot trial of infliximab, a chimeric monoclonal antibody to tumor necrosis factor-alpha, in patients with moderate-to-severe heart failure. Results of the anti-TNF therapy against congestive heart failure (ATTACH) trial.
  16. Effect of infliximab on sight-threatening panuveitis in Behcet’s disease.
  17. Evaluation of patients with scleritis for systemic disease.
  18. (1986). Experimental aspects and models of peripheral corneal disease. Int Ophthalmol Clin.
  19. Eye loss by exogenous endophthalmitis following anti-tumor necrosis factor therapy: a report of 4 cases.
  20. (1995). Fas ligand-induced apoptosis as a mechanism of immune privilege.
  21. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel.
  22. Immunoglobulins in the human cornea.
  23. (1998). Immunological and clinical evaluation of postsurgical necrotising sclerocorneal ulceration.
  24. Inflammatory diseases of the peripheral cornea.
  25. Inflammatory Terrien’s marginal corneal disease.
  26. Infliximab for progressive peripheral ulcerative keratitis in a patient with juvenile rheumatoid arthiritis.
  27. Infliximab for the treatment of refractory progressive sterile peripheral ulcerative keratitis associated with late corneal perforation: 3-year follow-up.
  28. (1994). Leflunomide: inhibition of S-antigen induced autoimmune uveitis in Lewis rats. Agents Actions.
  29. Mooren ulcer: an immunopathologic study.
  30. Mooren’s ulcer and amniotic membrane transplant: a simple surgical solution?
  31. (1992). Mooren’s ulcer and evidence of stromal graft rejection after penetrating keratoplasty.
  32. (2011). Mooren’s ulcer. In:
  33. (1996). Mooren’s ulcer. Int Ophthalmol Clin.
  34. Mortality rate in rheumatoid arthritis patients developing necrotizing scleritis or peripheral ulcerative keratitis: Effects of systemic immune suppression.
  35. Mycophenolate mofetil after methotrexate failure or intolerance in the treatment of scleritis and uveitis.
  36. (2002). Ocular characteristics and disease associations in scleritis-associated peripheral keratopathy. Arch Ophthalmol.
  37. Peripheral ulcerative keratitis ‘corneal melt’ and rheumatoid arthritis: a case series.
  38. (1998). Peripheral ulcerative keratitis and collagen vascular disease. Int Ophthalmol Clin.
  39. Peripheral ulcerative keratitis associated with vasculitis manifesting asymetrically as Fuchs superficial marginal keratitis and Terrien’s marginal dejeneration.
  40. (1996). Peripheral ulcerative keratitis in the collagen vascular diseases. Int Ophthalmol Clin.
  41. (2011). Phlyctenular keratoconjunctivitis and marginal staphylococcal keratitis.
  42. (2004). Recombinant human tumor necrosis factor receptor Fc fusion protein (Etanercept): experience as a therapy for sight-threatening scleritis and sterile corneal ulceration. Eye Contact Lens.
  43. Retinal vein thrombosis after infliximab (Remicade) treatment for Crohn’s disease.
  44. Retrospective analysis of children with uveitis treated with infliximab.
  45. Scleritis and peripheral ulcerative keratitis.
  46. (1994). Severity of scleritis and episcleritis.
  47. (2000). Systemic disorders associated with peripheral corneal ulceration. Curr Opin Ophthalmol.
  48. (1985). Systemic tetracyclines in the treatment of noninfected corneal ulcers: a case report and proposed new mechanism of action. Ann Ophthalmol.
  49. Tetracyclines and the treatment of corneal stromal ulceration: a review.
  50. (1991). Tetracyclines inhibit connective tissue breakdown: new therapeutic implications for an old family of drugs. Crit Rev Oral Biol Med.
  51. (1995). The human anion is a site of MHC class Ib expression: evidence for the expression of HLA-E
  52. The immunopathology of Mooren’s ulcer.
  53. (1971). The limbus. In:
  54. Therapy of progressive rheumatoid arthritis-associated corneal ulceration with infliximab.
  55. Three decades of corneal transplantation: indications and patient characteristics.
  56. Tumor necrosis factor a blockade with infliximab for refractory uveitis and scleritis.
  57. Tumor necrosis factor antagonists. Preliminary evidence for an emerging approach in the treatment of ocular inflammation.
  58. Twenty-five year panorama of corneal immunology.
  59. Use of infliximab in the treatment of peripheral ulcerative keratitis in Crohn’s disease.
  60. (1999). Vasculitic peripheral ulcerative keratitis. Surv Ophthalmol.
  61. Wegener’s granulomatosis: clinical manifestations, differential diagnosis, and management of ocular and systemic disease. Surv Ophthalmol.
  62. What is Mooren’s ulcer?

To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.