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Diagnosis and Management of Cicatricial Conjunctivitis

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Ocular Surface Disease

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Abstract

Cicatricial conjunctivitisis one of the most severe forms of ocular surface disease that can lead to profound loss of vision. The most critical aspect of the care is early recognition and the use of appropriate systemic immunomodulatory therapy. A common misconception is that if the conjunctival biopsy is negative for pemphigoid (i.e., no immunoglobulin deposition at the basement membrane), then it rules out the condition and it is not serious; however, false negatives are common and besides even true negative cases can be just as likely to progress and develop severe disease. Therefore, all cases of cicatricial conjunctivitis with active inflammation, regardless of biopsy results, require treatment to suppress inflammation. Another common misconception is that cicatricial conjunctival disease can be managed with local therapy, however which is rarely the case, and systemic therapy is indicated in nearly all cases. This chapter, contributed by the most preeminent expert and pioneer in this field, presents three cases with varying severity and highlights the latest diagnostic and management strategies.

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References

  1. Foster CS. Cicatricial pemphigoid. Trans Am Ophthalmol Soc. 1986;84:527–663.

    CAS  PubMed  PubMed Central  Google Scholar 

  2. Foster CS, Fong LP, Azar D, Kenyon KR. Episodic conjunctival inflammation after Stevens-Johnson syndrome. Ophthalmology. 1988;95(4):453–62.

    Article  CAS  PubMed  Google Scholar 

  3. Kirzhner M, Jakobiec FA. Ocular cicatricial pemphigoid: a review of clinical features, immunopathology, differential diagnosis, and current management. Semin Ophthalmol. 2011;26(4-5):270–7.

    Article  PubMed  Google Scholar 

  4. Ravage ZB, Beck AP, Macsai MS, Ching SS. Ocular rosacea can mimic trachoma: a case of cicatrizing conjunctivitis. Cornea. 2004;23(6):630–1.

    Article  PubMed  Google Scholar 

  5. Foster CS, Calonge M. Atopic keratoconjunctivitis. Ophthalmology. 1990;97(8):992–1000.

    Article  CAS  PubMed  Google Scholar 

  6. Geggel HS, Mensher JH. Cicatricial conjunctivitis in sarcoidosis: recognition and treatment. Ann Ophthalmol. 1989;21(3):92–4.

    CAS  PubMed  Google Scholar 

  7. Nguyen DQ, Harper J, Hiscott P, Quah SA, Jacob A, Kaye SB. The significance of cicatricial conjunctivitis in Wegener’s granulomatosis. Nephrol Dial Transplant. 2006;21(11):3342–3.

    Article  PubMed  Google Scholar 

  8. Miserocchi E, Waheed NK, Baltatzis S, Foster CS. Chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and fatal Wegener granulomatosis. Am J Ophthalmol. 2001;132(6):923–4.

    Article  CAS  PubMed  Google Scholar 

  9. Ahuero AE, Jakobiec FA, Bhat P, Ciralsky JB, Papaliodis GN. Paraneoplastic conjunctival cicatrization: two different pathogenic types. Ophthalmology. 2010;117(4):659–64.

    Article  PubMed  Google Scholar 

  10. Taylor HR, Burton MJ, Haddad D, West S, Wright H. Trachoma. Lancet. 2014;384(9960):2142–52.

    Article  PubMed  Google Scholar 

  11. Foster CS, Sainz De La Maza M. Ocular cicatricial pemphigoid review. Curr Opin Allergy Clin Immunol. 2004;4(5):435–9.

    Google Scholar 

  12. Faraj HG, Hoang-Xuan T. Chronic cicatrizing conjunctivitis. Curr Opin Ophthalmol. 2001;12(4):250–7.

    Article  CAS  PubMed  Google Scholar 

  13. Eschle-Meniconi ME, Ahmad SR, Foster CS. Mucous membrane pemphigoid: an update. Curr Opin Ophthalmol. 2005;16(5):303–7.

    Article  PubMed  Google Scholar 

  14. Bernauer W, Elder MJ, Leonard JN, Wright P, Dart JK. The value of biopsies in the evaluation of chronic progressive conjunctival cicatrisation. Graefes Arch Clin Exp Ophthalmol. 1994;232(9):533–7.

    Article  CAS  PubMed  Google Scholar 

  15. Schempf U, Plentz R, Stueker D, et al. Scarring mucous membrane pemphigoid presenting as double stenosis of the larynx and esophagus: precautions during therapy can avoid complications. Endoscopy. 2014;46(Suppl 1):E617–8.

    PubMed  Google Scholar 

  16. Grau AE, Setterfield J, Saw VP. How to do conjunctival and buccal biopsies to investigate cicatrising conjunctivitis: improving the diagnosis of ocular mucous membrane pemphigoid. Br J Ophthalmol. 2013;97(4):530–1.

    Article  PubMed  Google Scholar 

  17. Saw VP, Dart JK, Rauz S, et al. Immunosuppressive therapy for ocular mucous membrane pemphigoid strategies and outcomes. Ophthalmology. 2008;115(2):253–61, e251.

    Google Scholar 

  18. McCluskey P, Chang JH, Singh R, Wakefield D. Methotrexate therapy for ocular cicatricial pemphigoid. Ophthalmology. 2004;111(4):796–801.

    Article  PubMed  Google Scholar 

  19. Miserocchi E, Baltatzis S, Roque MR, Ahmed AR, Foster CS. The effect of treatment and its related side effects in patients with severe ocular cicatricial pemphigoid. Ophthalmology. 2002;109(1):111–8.

    Article  PubMed  Google Scholar 

  20. Geerling G, Dart JK. Management and outcome of cataract surgery in ocular cicatricial pemphigoid. Graefes Arch Clin Exp Ophthalmol. 2000;238(2):112–8.

    Article  CAS  PubMed  Google Scholar 

  21. Puranik CJ, Murthy SI, Taneja M, Sangwan VS. Outcomes of cataract surgery in ocular cicatricial pemphigoid. Ocul Immunol Inflamm. 2013;21(6):449–54.

    Article  PubMed  Google Scholar 

  22. Bissen-Miyajima H, Monden Y, Shimazaki J, Tsubota K. Cataract surgery combined with ocular surface reconstruction in patients with severe cicatricial keratoconjunctivitis. J Cataract Refract Surg. 2002;28(8):1379–85.

    Article  PubMed  Google Scholar 

  23. Sainz de la Maza M, Tauber J, Foster CS. Cataract surgery in ocular cicatricial pemphigoid. Ophthalmology. 1988;95(4):481–6.

    Article  CAS  PubMed  Google Scholar 

  24. Foster CS, Chang PY, Ahmed AR. Combination of rituximab and intravenous immunoglobulin for recalcitrant ocular cicatricial pemphigoid: a preliminary report. Ophthalmology. 2010;117(5):861–9.

    Article  PubMed  Google Scholar 

  25. Sami N, Letko E, Androudi S, Daoud Y, Foster CS, Ahmed AR. Intravenous immunoglobulin therapy in patients with ocular-cicatricial pemphigoid: a long-term follow-up. Ophthalmology. 2004;111(7):1380–2.

    Article  PubMed  Google Scholar 

  26. Sobolewska B, Doycheva D, Deuter C, Pfeffer I, Schaller M, Zierhut M. Treatment of ocular rosacea with once-daily low-dose doxycycline. Cornea. 2014;33(3):257–60.

    Article  PubMed  Google Scholar 

  27. Suelves AM, Arcinue CA, Gonzalez-Martin JM, Kruh JN, Foster CS. Analysis of a novel protocol of pulsed intravenous cyclophosphamide for recalcitrant or severe ocular inflammatory disease. Ophthalmology. 2013;120(6):1201–9.

    Article  PubMed  Google Scholar 

  28. Friedman J, Marcovich AL, Kleinmann G, Schattner A. Low-dose pulsed intravenous cyclophosphamide for severe ocular cicatricial pemphigoid in elderly patients. Cornea. 2014;33(10):1066–70.

    Article  PubMed  Google Scholar 

  29. Tugal-Tutkun I, Akova YA, Foster CS. Penetrating keratoplasty in cicatrizing conjunctival diseases. Ophthalmology. 1995;102(4):576–85.

    Article  CAS  PubMed  Google Scholar 

  30. Palioura S, Kim B, Dohlman CH, Chodosh J. The Boston keratoprosthesis type I in mucous membrane pemphigoid. Cornea. 2013;32(7):956–61.

    Article  PubMed  Google Scholar 

  31. Pujari S, Siddique SS, Dohlman CH, Chodosh J. The Boston keratoprosthesis type II: the Massachusetts eye and ear infirmary experience. Cornea. 2011;30(12):1298–303.

    Article  PubMed  Google Scholar 

  32. Ahmad S, Akpek EK, Gehlbach PL, Dunlap K, Ramulu PY. Predictors of visual outcomes following Boston type 1 keratoprosthesis implantation. Am J Ophthalmol. 2015;159(4):739–47.

    Article  PubMed  Google Scholar 

  33. Sotozono C, Inatomi T, Nakamura T, et al. Visual improvement after cultivated oral mucosal epithelial transplantation. Ophthalmology. 2013;120(1):193–200.

    Article  PubMed  Google Scholar 

  34. Ahmed AR, Dahl MV. Consensus statement on the use of intravenous immunoglobulin therapy in the treatment of autoimmune mucocutaneous blistering diseases. Arch Dermatol. 2003;139(8):1051–9.

    Article  CAS  PubMed  Google Scholar 

  35. Power WJ, Tugal-Tutkun I, Foster CS. Long-term follow-up of patients with atopic keratoconjunctivitis. Ophthalmology. 1998;105(4):637–42.

    Article  CAS  PubMed  Google Scholar 

  36. Wakamatsu TH, Satake Y, Igarashi A, et al. IgE and eosinophil cationic protein (ECP) as markers of severity in the diagnosis of atopic keratoconjunctivitis. Br J Ophthalmol. 2012;96(4):581–6.

    Article  PubMed  Google Scholar 

  37. Arita R, Morishige N, Koh S, et al. Increased tear fluid production as a compensatory response to meibomian gland loss: a multicenter cross-sectional study. Ophthalmology. 2015;122(5):925–33.

    Article  PubMed  Google Scholar 

  38. Matsumoto Y, Dogru M, Goto E, et al. Alterations of the tear film and ocular surface health in chronic smokers. Eye. 2008;22(7):961–8.

    Article  CAS  PubMed  Google Scholar 

  39. Schornack MM, Baratz KH. Ocular cicatricial pemphigoid: the role of scleral lenses in disease management. Cornea. 2009;28(10):1170–2.

    Article  PubMed  Google Scholar 

  40. Gibbons A, Johnson TE, Wester ST, et al. Management of patients with confirmed and presumed mucous membrane pemphigoid undergoing entropion repair. Am J Ophthalmol. 2015;159(5):846–52, e842.

    Google Scholar 

  41. Nivenius E, van der Ploeg I, Jung K, Chryssanthou E, van Hage M, Montan PG. Tacrolimus ointment vs steroid ointment for eyelid dermatitis in patients with atopic keratoconjunctivitis. Eye. 2007;21(7):968–75.

    Article  CAS  PubMed  Google Scholar 

  42. Gonzalez-Lopez JJ, Lopez-Alcalde J, Morcillo Laiz R, Fernandez Buenaga R, Rebolleda Fernandez G. Topical cyclosporine for atopic keratoconjunctivitis. Cochrane Database Syst Rev. 2012;9:CD009078.

    Google Scholar 

  43. Tesse R, Spadavecchia L, Fanelli P, et al. Treatment of severe vernal keratoconjunctivitis with 1% topical cyclosporine in an Italian cohort of 197 children. Pediatr Allergy Immunol. 2010;21(2 Pt 1):330–5.

    Article  PubMed  Google Scholar 

  44. Hatton MP, Raizman M, Foster CS. Exacerbation of undiagnosed ocular cicatricial pemphigoid after repair of involutional entropion. Ophthal Plast Reconstr Surg. 2008;24(2):165–6.

    Article  PubMed  Google Scholar 

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Acknowledgments

Financial Disclosures: None of the authors have any financial disclosures regarding the contents discussed in this manuscript. Conflict of Interest: None of the authors have any conflicts of interest with the contents discussed in this manuscript.

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Authors and Affiliations

  1. Massachusetts Eye Research and Surgery Institution (MERSI), 1440 Main Street, Suite 201, Waltham, MA, 02451, USA

    Robert T. Swan MD, Jennifer Cao MD & C. Stephen Foster MD, FACS

  2. Ocular Immunology and Uveitis Foundation, Waltham, MA, USA

    Robert T. Swan MD, Jennifer Cao MD & C. Stephen Foster MD, FACS

  3. Harvard Medical School, Boston, MA, USA

    C. Stephen Foster MD, FACS

Authors
  1. Robert T. Swan MD
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  2. Jennifer Cao MD
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  3. C. Stephen Foster MD, FACS
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Corresponding author

Correspondence to C. Stephen Foster MD, FACS .

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Editors and Affiliations

  1. Cornea Service, Illinois Eye and Ear Infirmary, Chicago, Illinois, USA

    Ali R. Djalilian

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Swan, R.T., Cao, J., Foster, C.S. (2018). Diagnosis and Management of Cicatricial Conjunctivitis. In: Djalilian, A. (eds) Ocular Surface Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-15823-5_12

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  • DOI: https://doi.org/10.1007/978-3-319-15823-5_12

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-15822-8

  • Online ISBN: 978-3-319-15823-5

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