Skip to main content

Clinical characteristics of scleritis patients with emphasized comparison of associated systemic diseases (anti-neutrophil cytoplasmic antibody-associated vasculitis and rheumatoid arthritis)

Japanese Journal of Ophthalmology volume 63, pages 417–424 (2019)Cite this article

Abstract

Purpose

We evaluated patient profiles, clinical features, associated systemic diseases, treatment modalities, and ocular complications in cases of scleritis and episcleritis.

Study design

Retrospective.

Methods

Clinical data of 128 patients referred to the ophthalmology clinic at Jichi Medical University Hospital during the 4-year period from April 2011 to March 2015, and diagnosed with scleritis or episcleritis were examined. Gender, average onset age, unilateral or bilateral manifestation, classification type, associated systemic diseases, and treatments were retrospectively investigated.

Results

The cohort consisted of 57 men and 71 women. Average onset age was 54.3 ± 17.4 years. Diffuse anterior scleritis was the most common type. It was noted in 43 (32.8%) patients, followed by episcleritis in 35 (27.3%), nodular anterior scleritis in 23 (18.0%), necrotizing anterior scleritis in 22 (17.2%), and posterior scleritis in 6 (4.7%). Eighteen (81.8%) of 22 patients with necrotizing anterior scleritis required some type of systemic medication, including corticosteroid, cyclophosphamide, cyclosporine, azathioprine, methotrexate, or rituximab administration. Forty (31.3%) had associated systemic diseases, which included 10 with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and 8 with rheumatoid arthritis (RA). Patients with ANCA-associated vasculitis had a tendency to develop scleritis first and had significantly worse visual prognoses compared to those with RA.

Conclusions

Approximately 30% of the patients with scleritis and episcleritis had complications involving systemic diseases, including ten patients with ANCA-associated vasculitis and 8 with RA. ANCA-associated vasculitis was more often diagnosed after scleritis and patients suffered poorer visual prognoses than those with RA.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

43,34 €

Price includes VAT (Finland)
Tax calculation will be finalised during checkout.

References

  1. Okhravi N, Odufuwa B, McCluskey P, Lightman S. Scleritis. Surv Ophthalmol. 2005;50:351–63.

    Article  PubMed  Google Scholar 

  2. Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol. 1976;60:163–91.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Akpek EK, Throne EJ, Qazi FA, Do DV, Jabs DA. Evaluation of patients with scleritis for systemic disease. Ophthalmology. 2004;111:501–6.

    Article  PubMed  Google Scholar 

  4. McGavin DD, Williamson J, Forrester JV, Foulds WS, Buchanan WW, Dick WC, et al. Episcleritis and scleritis. A study of their clinical manifestation and association with rheumatoid arthritis. Br J Ophthalmol. 1976;60:192–226.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. JCS Joint Working Group. Guideline for management of vasculitis syndrome (JCS 2008). Japanese Circulation Society. Circ J. 2011;75:474–503.

    Article  Google Scholar 

  6. Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaître O, Cohen P, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371:1771–80.

    Article  CAS  PubMed  Google Scholar 

  7. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363:221–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Tanaka R, Kaburaki T, Ohtomo K, Takamoto M, Komae K, Numaga J, et al. Clinical characteristics and ocular complications of patients with scleritis in Japanese. Jpn J Ophthalmol. 2018;62:517–24.

    Article  CAS  PubMed  Google Scholar 

  9. Ohguro N, Sonoda K, Takeuchi M, Matsumura M, Mochizuki M. The 2009 prospective multi-center epidemiologic survey of uveitis in Japan. Jpn J Ophthalmol. 2012;56:432–5.

    Article  PubMed  Google Scholar 

  10. Keino H, Watanabe T, Taki W, Nakashima C, Okada AA. Clinical features and visual outcomes of Japanese patients with scleritis. Br J Ophthalmol. 2010;94:1459–63.

    Article  CAS  PubMed  Google Scholar 

  11. Berichicci L, Miserocchi E, Di Nicola M, La Spina C, Bandello F, Modorati G. Clinical features of patients with episcleritis and scleritis in an Italian tertiary care referral center. Eur J Ophthalmol. 2014;24:293–8.

    Article  Google Scholar 

  12. Gonzalez-Gonzalez LA, Molina-Prat N, Doctor P, Tauber J, de la Maza SM, Foster CS. Clinical features and presentation of posterior scleritis: a report of 31 cases. Ocul Immunol Inflamm. 2014;22:203–7.

    Article  PubMed  Google Scholar 

  13. Oono S, Kurimoto T, Oku H, Mimura O. Adult T-cell leukemia presenting with episcleritis and secondary glaucoma. Jpn J Ophthalmol. 2009;53:70–1.

    Article  PubMed  Google Scholar 

  14. Smith LK, Suhler EB, Lim LL, Choi D, Cioffi GA, Rosenbaum JT. Possible association between scleritis and lymphoma. Br J Ophthalmol. 2007;91:1728–9.

    Article  PubMed  Google Scholar 

  15. Hoang LT, Lim LL, Vaillant B, Choi D, Rosenbaum JT. Antineutrophil cytoplamic antibody-associated active scleritis. Arch Ophthalmol. 2008;126:651–5.

    Article  PubMed  Google Scholar 

  16. Lin P, Bhullar SS, Tessler HH, Goldstein DA. Immunologic markers as potential predictors of systemic autoimmune disease in patients with idiopathic scleritis. Am J Ophthalmol. 2008;145:463–71.

    Article  CAS  PubMed  Google Scholar 

  17. Kuroyanagi K, Sakai T, Yoshida K, Yamada A, Shiba T, Tsuneoka H. Necrotizing scleritis in a patient positive for both c- and p-ANCA without underlying systemic vasculitis. Jpn J Ophthalmol. 2011;55:581–2.

    Article  PubMed  Google Scholar 

  18. Watkins AS, Kempen JH, Choi D, Liesegang TL, Pujari SS, Newcomb C, et al. Ocular disease in patients with ANCA-positive vasculitis. J Ocul Biol Dis Inform. 2010;3:12–9.

    Article  Google Scholar 

  19. de la Maza SM, Foster CS, Jabbur NS. Scleritis associated with systemic vasculitic diseases. Ophthalmology. 1995;102:687–92.

    Article  Google Scholar 

  20. Alrashidi S, Yousef YA, Krema H. Visaul loss from scleritis in C-ANCA microscopic polyangiitis. BMJ Case Rep. 2013;10:bcr2013009583.

    Google Scholar 

  21. de la Maza SM, Molina N, Gonzalez-Gonzalez LA, Doctor PP, Tauber J, Foster CS. Scleritis therapy. Ophthalmology. 2012;119:51–8.

    Article  Google Scholar 

  22. de la Maza SM, Jabbur NS, Foster CS. Severity of scleritis and episcleritis. Ophthalmology. 1994;101:389–96.

    Article  Google Scholar 

  23. Suhler EB, Lim LL, Beardsley RM, Giles TR, Pasadhika S, Lee ST, et al. Rituximab therapy for refractory scleritis: results of a phase I/II dose-ranging, randomized, clinical trial. Ophthalmology. 2014;121:1885–91.

    Article  PubMed  Google Scholar 

  24. McCluskey P, Wakefield D. Prediction of response to treatment in patients with scleritis using a standardised scoring system. Aust N Z J Ophthalmol. 1991;19:211–5.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Affiliations

  1. Division of Ophthalmology, The Cancer Institute Hospital of JFCR, 3-8-31 Ariake, Koto, Tokyo, 135-8550, Japan

    Atsushi Yoshida

  2. Department of Ophthalmology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan

    Atsushi Yoshida, Meri Watanabe, Akira Okubo & Hidetoshi Kawashima

  3. Okubo Eye Clinic, 1137-4 Hiramatsu-honcho, Utsunomiya, Tochigi, 321-0932, Japan

    Akira Okubo

Authors
  1. Atsushi Yoshida
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Meri Watanabe
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Akira Okubo
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Hidetoshi Kawashima
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Atsushi Yoshida.

Ethics declarations

Conflicts of interest

A. Yoshida, None; M. Watanabe, None; A. Okubo, None; H. Kawashima, None.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Corresponding author: Atsushi Yoshida

About this article

Verify currency and authenticity via CrossMark

Cite this article

Yoshida, A., Watanabe, M., Okubo, A. et al. Clinical characteristics of scleritis patients with emphasized comparison of associated systemic diseases (anti-neutrophil cytoplasmic antibody-associated vasculitis and rheumatoid arthritis). Jpn J Ophthalmol 63, 417–424 (2019). https://doi.org/10.1007/s10384-019-00674-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10384-019-00674-7

Keywords

  • Scleritis
  • Episcleritis
  • Rheumatoid arthritis
  • Anti-neutrophil cytoplasmic antibody (ANCA)
  • ANCA-associated vasculitis