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

  • Atsushi YoshidaEmail author
  • Meri Watanabe
  • Akira Okubo
  • Hidetoshi Kawashima
Clinical Investigation



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

Study design



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.


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.


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.


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


Conflicts of interest

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


  1. 1.
    Okhravi N, Odufuwa B, McCluskey P, Lightman S. Scleritis. Surv Ophthalmol. 2005;50:351–63.CrossRefGoogle Scholar
  2. 2.
    Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol. 1976;60:163–91.CrossRefGoogle Scholar
  3. 3.
    Akpek EK, Throne EJ, Qazi FA, Do DV, Jabs DA. Evaluation of patients with scleritis for systemic disease. Ophthalmology. 2004;111:501–6.CrossRefGoogle Scholar
  4. 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.CrossRefGoogle Scholar
  5. 5.
    JCS Joint Working Group. Guideline for management of vasculitis syndrome (JCS 2008). Japanese Circulation Society. Circ J. 2011;75:474–503.CrossRefGoogle Scholar
  6. 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.CrossRefGoogle Scholar
  7. 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.CrossRefGoogle Scholar
  8. 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.CrossRefGoogle Scholar
  9. 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.CrossRefGoogle Scholar
  10. 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.CrossRefGoogle Scholar
  11. 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.CrossRefGoogle Scholar
  12. 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.CrossRefGoogle Scholar
  13. 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.CrossRefGoogle Scholar
  14. 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.CrossRefGoogle Scholar
  15. 15.
    Hoang LT, Lim LL, Vaillant B, Choi D, Rosenbaum JT. Antineutrophil cytoplamic antibody-associated active scleritis. Arch Ophthalmol. 2008;126:651–5.CrossRefGoogle Scholar
  16. 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.CrossRefGoogle Scholar
  17. 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.CrossRefGoogle Scholar
  18. 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.CrossRefGoogle Scholar
  19. 19.
    de la Maza SM, Foster CS, Jabbur NS. Scleritis associated with systemic vasculitic diseases. Ophthalmology. 1995;102:687–92.CrossRefGoogle Scholar
  20. 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. 21.
    de la Maza SM, Molina N, Gonzalez-Gonzalez LA, Doctor PP, Tauber J, Foster CS. Scleritis therapy. Ophthalmology. 2012;119:51–8.CrossRefGoogle Scholar
  22. 22.
    de la Maza SM, Jabbur NS, Foster CS. Severity of scleritis and episcleritis. Ophthalmology. 1994;101:389–96.CrossRefGoogle Scholar
  23. 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.CrossRefGoogle Scholar
  24. 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.CrossRefGoogle Scholar

Copyright information

© Japanese Ophthalmological Society 2019

Authors and Affiliations

  • Atsushi Yoshida
    • 1
    • 2
    Email author
  • Meri Watanabe
    • 2
  • Akira Okubo
    • 2
    • 3
  • Hidetoshi Kawashima
    • 2
  1. 1.Division of OphthalmologyThe Cancer Institute Hospital of JFCRTokyoJapan
  2. 2.Department of OphthalmologyJichi Medical UniversityShimotsukeJapan
  3. 3.Okubo Eye ClinicUtsunomiyaJapan

Personalised recommendations