Skip to main content
SpringerLink
Log in

Noninfectious Causes

  • Chapter
  • First Online:
Peripheral Ulcerative Keratitis

Part of the book series: Essentials in Ophthalmology ((ESSENTIALS))

  • 743 Accesses

Abstract

Peripheral ulcerative keratitis (PUK) can result as a consequence of a variety of noninfectious inflammatory diseases. Inflammatory PUK is usually a result of an autoimmune destruction of the perilimbal cornea, most often from a connective tissue disease, but it can occur from other inflammatory processes as well. Peripheral ulcerative corneal involvement from a connective tissue disorder can often be a sentinel sign of the presence and progression of a systemic, potentially life-threatening vasculitis. Appropriate management of PUK includes control of systemic inflammation, as well as preservation of both corneal and globe integrity. First-line immunosuppressive therapy with systemic corticosteroid may need to be supplemented by cytotoxic and/or immunomodulatory agents to prevent disease progression and stabilize the ocular surface. The new biologic agents, such as the TNF-alpha inhibitor infliximab and rituximab, have also proven effective in refractory cases of PUK. The potential side effects of immunosuppressive therapy and the systemic involvement of the inflammatory process in PUK require a multidisciplinary approach between ophthalmologists, internists, and rheumatologists for both diagnosis and management of these conditions.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 54.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Messmer EM, Foster CS. Vasculitic peripheral ulcerative keratitis. Surv Ophthalmol. 1999;43(5):379–96.

    Article  CAS  PubMed  Google Scholar 

  2. Tauber J, Sainz de la Maza M, Hoang-Xuan T, Foster CS. An analysis of therapeutic decision making regarding immunosuppressive chemotherapy for peripheral ulcerative keratitis. Cornea. 1990;9(1):66–73.

    Article  CAS  PubMed  Google Scholar 

  3. Sainz de la Maza M, Foster CS, Jabbur NS, Baltatzis S. Ocular characteristics and disease associations in scleritis-associated peripheral keratopathy. Arch Ophthalmol. 2002;120(1):15–9.

    Article  PubMed  Google Scholar 

  4. Gregory JK, Foster CS. Peripheral ulcerative keratitis in the collagen vascular diseases. Int Ophthalmol Clin. 1996;36(1):21–30.

    Article  CAS  PubMed  Google Scholar 

  5. Ladas JG, Mondino BJ. Systemic disorders associated with peripheral corneal ulceration. Curr Opin Ophthalmol. 2000;11(6):468–71.

    Article  CAS  PubMed  Google Scholar 

  6. Riley GP, Harrall RL, Watson PG, Cawston TE, Hazleman BL. Collagenase (MMP-1) and TIMP-1 in destructive corneal disease associated with rheumatoid arthritis. Eye (Lond). 1995;9(6):703–18. doi:10.1038/eye.1995.182.

    Article  Google Scholar 

  7. 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(10):1253–63.

    Article  CAS  PubMed  Google Scholar 

  8. Thomas JW, Pflugfelder SC. Therapy of progressive rheumatoid arthritis-associated corneal ulceration with infliximab. Cornea. 2005;24(6):742–4.

    Article  PubMed  Google Scholar 

  9. Watanabe R, Ishii T, Yoshida M, Takada N, Yokokura S, Shirota Y, Fujii H, Harigae H. Ulcerative keratitis in patients with rheumatoid arthritis in the modern biologic era: A series of eight cases and literature review. Int J Rheum Dis. 2015. doi:10.1111/1756-185X.12688.

  10. Freidlin J, Wong IG, Acharya N. Rituximab treatment for peripheral ulcerative keratitis associated with Wegener’s granulomatosis. Br J Ophthalmol. 2007;91(10):1414. doi:10.1136/bjo.2006.113316.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Huerva V, Sanchez MC, Traveset A, Jurjo C, Ruiz A. Rituximab for peripheral ulcerative keratitis with wegener granulomatosis. Cornea. 2010;29(6):708–10. doi:10.1097/ICO.0b013e3181c296ed.

    Article  PubMed  Google Scholar 

  12. Leib ES, Restivo C, Paulus HE. Immunosuppressive and corticosteroid therapy of polyarteritis nodosa. Am J Med. 1979;67(6):941–7.

    Article  CAS  PubMed  Google Scholar 

  13. Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C. Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford). 2007;46(12):1757–62. doi:10.1093/rheumatology/kem173.

    Article  CAS  Google Scholar 

  14. Read RW. Clinical mini-review: Systemic lupus erythematosus and the eye. Ocul Immunol Inflamm. 2004;12(2):87–99. doi:10.1080/09273940490895308.

    Article  PubMed  Google Scholar 

  15. Yoo JH, Chodosh J, Dana R. Relapsing polychondritis: systemic and ocular manifestations, differential diagnosis, management, and prognosis. Semin Ophthalmol. 2011;26(4–5):261–9. doi:10.3109/08820538.2011.588653.

    Article  PubMed  Google Scholar 

  16. Jabbarvand M, Fard MA. Infliximab in a patient with refractory necrotizing scleritis associated with relapsing polychondritis. Ocular immunology and inflammation. 2010;18(3):216–7.

    Article  CAS  PubMed  Google Scholar 

  17. Siracuse-Lee D, Saffra N. Peripheral ulcerative keratitis in sarcoidosis: A case report. Cornea. 2006;25(5):618–20.

    Article  PubMed  Google Scholar 

  18. Harthan JS, Reeder RE. Peripheral ulcerative keratitis in association with sarcoidosis. Contact Lens Anterior Eye. 2013;36(6):313–7.

    Article  PubMed  Google Scholar 

  19. Tan MH, Chen SDM, Rubinstein A, Bron AJ. Corneal perforation due to severe peripheral ulcerative keratitis in Crohn disease. Cornea. 2006;25(5):628–30.

    Article  PubMed  Google Scholar 

  20. Angioi K, Kaminsky P, Peyrin-Biroulet L. Infliximab for severe peripheral ulcerative keratopathy revealing Crohn’s disease. Inflamm Bowel Dis. 2011;17 (3):866–867. doi:10.1002/ibd.21358.

  21. Pham M, Chow CC, Badawi D, Tu EY. Use of infliximab in the treatment of peripheral ulcerative keratitis in Crohn disease. Am J Ophthalmol. 2011;152(2):183–188.e182.

    Google Scholar 

  22. Angioi K, Kaminsky P, Peyrin-Biroulet L. Infliximab for severe peripheral ulcerative keratopathy revealing Crohn’s disease. Inflamm Bowel Dis. 2011;17(3):866–867. doi:10.1002/ibd.21358.

  23. Zegans ME, Srinivasan M. Mooren’s ulcer. Int Ophthalmol Clin. 1998;38(4):81–8.

    Article  CAS  PubMed  Google Scholar 

  24. Watson PG. Management of Mooren’s ulceration. Eye (Lond). 1997;11(Pt 3):349–56. doi:10.1038/eye.1997.74.

    Article  Google Scholar 

  25. Alhassan MB, Rabiu M, Agbabiaka IO. Interventions for Mooren’s ulcer. The Cochrane database of systematic reviews (6). doi:10.1002/14651858.CD006131.pub2.

  26. Sangwan VS, Zafirakis P, Foster CS. Mooren’s ulcer: Current concepts in management. Indian J Ophthalmol. 1997;45(1):7–17.

    CAS  PubMed  Google Scholar 

  27. Wilson SE, Lee WM, Murakami C, Weng J, Moninger GA. Mooren-type hepatitis C virus-associated corneal ulceration. Ophthalmology. 1994;101(4):736–45.

    Article  CAS  PubMed  Google Scholar 

  28. Ozcura F. Successful treatment of staphylococcus-associated marginal keratitis with topical cyclosporine. Graefes Arch Clin Exp Ophthalmol. 2010;248(7):1049–50. doi:10.1007/s00417-009-1290-4.

    Article  PubMed  Google Scholar 

  29. Bierly JR, Dunn JP, Dawson CR, Ostler HB, Wong IG. Fuchs’ superficial marginal keratitis. Am J Ophthalmol. 1992;113(5):541–5.

    Article  CAS  PubMed  Google Scholar 

  30. Goldberg MA, Lubniewski AJ, Williams JM, Smith ME, Pepose JS. Cystic hydrops and spontaneous perforation in Fuchs’ superficial marginal keratitis. Am J Ophthalmol. 1996;121(1):91–3.

    Article  CAS  PubMed  Google Scholar 

  31. Mejia LF, Santamaria JP, Gaviria AM, Rodriguez AM. Fuchs’ superficial marginal keratitis managed with circumferential marginal corneoscleral lamellar patch graft. Eur J Ophthalmol. 2013;23(6):925–7. doi:10.5301/ejo.5000356.

    Article  PubMed  Google Scholar 

  32. Galor A, Thorne JE. Scleritis and peripheral ulcerative keratitis. Rheum Dis Clin North Am. 2007;33(4):835–854, vii. doi:10.1016/j.rdc.2007.08.002.

  33. Galor A, Jabs DA, Leder HA, Kedhar SR, Dunn JP, Peters GB 3rd, Thorne JE. Comparison of antimetabolite drugs as corticosteroid-sparing therapy for noninfectious ocular inflammation. Ophthalmology. 2008;115(10):1826–32. doi:10.1016/j.ophtha.2008.04.026.

    Article  PubMed  Google Scholar 

  34. Jabs DA, Rosenbaum JT, Foster CS, Holland GN, Jaffe GJ, Louie JS, Nussenblatt RB, Stiehm ER, Tessler H, Van Gelder RN, Whitcup SM, Yocum D. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: Recommendations of an expert panel. Am J Ophthalmol. 2000;130(4):492–513.

    Article  CAS  PubMed  Google Scholar 

  35. Cuchacovich M, Solanes F, Perez C, Verdaguer JI, Verdaguer J, Castiglione E, Carpentier C, Traipe L, Villarroel F. Mycophenolate mofetil therapy in refractory inflammatory eye disease. J Ocul Pharmacol Ther. 2015;. doi:10.1089/jop.2015.0044.

    PubMed  Google Scholar 

  36. Sobrin L, Christen W, Foster CS. Mycophenolate mofetil after methotrexate failure or intolerance in the treatment of scleritis and uveitis. Ophthalmology. 2008; 115(8):1416–1421, 1421 e1411. doi:10.1016/j.ophtha.2007.12.011.

  37. Jia Y, Gao H, Li S, Shi W. Combined anterior chamber washout, amniotic membrane transplantation, and topical use of corticosteroids for severe peripheral ulcerative keratitis. Cornea. 2014;33(6):559–64. doi:10.1097/ico.0000000000000130.

    Article  PubMed  Google Scholar 

  38. Yagci A. Update on peripheral ulcerative keratitis. Clin Ophthalmol. 2012;6:747–54. doi:10.2147/opth.s24947.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Maya JR, Sadiq MA, Zapata LJ, Hanout M, Sarwar S, Rajagopalan N, Guinn KE, Sepah YJ, Nguyen QD. Emerging therapies for noninfectious uveitis: what may be coming to the clinics. J Ophthalmol. 2014;2014:310329. doi:10.1155/2014/310329.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Compliance with Ethical Requirements

Conflict of Interest

Jessica Chow and Vincent de Luise declare that they have no conflict of interest.

Informed Consent

No human studies were carried out by the authors for this article.

Animal Studies

No animal studies were carried out by the authors for this article.

Author information

Authors and Affiliations

  1. Department of Ophthalmology and Visual Science, Yale University, 40 Temple Street, Suite 3D, New Haven, CT, 06510, USA

    Jessica Chow

  2. Department of Ophthalmology, Yale University School of Medicine, PO Box 112, Woodbury, CT, 06798, USA

    Vincent P. deLuise

Authors
  1. Jessica Chow
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Vincent P. deLuise
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jessica Chow .

Editor information

Editors and Affiliations

  1. Department of Cornea and Refractive Services, Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, Delhi, India

    Radhika Tandon

  2. Department of Ophthalmology, Clinical Ophthalmology, Miami VAMC, Bascom Palmer Eye Institute, University of Miami, Miami, Florida, USA

    Anat Galor

  3. Clinical Trial Center, L V Prasad Eye Institute, Hyderabad, India

    Virender Singh Sangwan

  4. Department of Ophthalmology, National University Hospital, Singapore, Singapore and, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore

    Manotosh Ray

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing AG

About this chapter

Cite this chapter

Chow, J., deLuise, V.P. (2017). Noninfectious Causes. In: Tandon, R., Galor, A., Sangwan, V., Ray, M. (eds) Peripheral Ulcerative Keratitis. Essentials in Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-319-50404-9_9

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-50404-9_9

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-50402-5

  • Online ISBN: 978-3-319-50404-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation