Scleritis Treatment & Management
- Author: Maite Sainz de la Maza, MD, PhD; Chief Editor: Hampton Roy Sr, MD more...
Medical Care
Treatment of scleritis almost always requires systemic therapy. Patients with an associated disease, such as rosacea, gout, atopy, or infection, need specific treatment.
- Treatment of noninfectious scleritis: Nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, or immunomodulatory drugs are indicated. Topical therapy is routinely insufficient. This treatment must be individualized for the severity of the scleritis, response to treatment, adverse effects, and presence of the associated disease.
- Diffuse scleritis or nodular scleritis
- The initial therapy consists of an NSAID; in case of therapeutic failure, 2 different NSAIDs should be tried in succession with the first drug. In high-risk patients, consider appropriate gastrointestinal protection with misoprostol or omeprazole.
- If NSAIDs are not effective or have untoward complications, oral corticosteroids can be substituted. Remission may be maintained with continued NSAIDs.
- Periorbital and subconjunctival steroid injections have been reported to be efficacious as adjunctive therapy. Caution should be observed.[6]
- In case of therapeutic failure of corticosteroids, immunosuppressive drugs should be added or substituted. Methotrexate (MTX) can be the first choice, but azathioprine, mycophenolate mofetil, cyclophosphamide, or cyclosporine may be helpful.[7, 8, 9] Cyclophosphamide should be the first choice in treating patients with associated potentially lethal vasculitic diseases, such as Wegener granulomatosis or polyarteritis nodosa.
- In case of therapeutic failure, other immunomodulatory drugs, such as infliximab or adalimumab, may be effective.[10, 11, 12, 13] Other alternatives are daclizumab and rituximab, although further investigation is warranted.[14, 15, 16]
- Necrotizing scleritis
- The initial therapy consists of immunosuppressive drugs that are supplemented with corticosteroids during the first month; the latter is tapered slowly, if possible. Cyclophosphamide should be the first choice in treating patients with an underlying systemic vasculitis such as Wegener granulomatosis or polyarteritis nodosa
- In case of therapeutic failure, other immunomodulatory drugs, such as infliximab or adalimumab, may be effective. Other alternatives are daclizumab and rituximab, although their efficacy awaits further study.[16, 17]
- Periocular steroid injections should not be applied in cases of necrotizing scleritis or peripheral ulcerative keratitis. Some authors believe that depot steroids actually may exacerbate necrotizing disease.
- Pulse intravenous cyclophosphamide with or without pulse intravenous corticosteroids may be required for urgent cases and may be followed by maintenance therapy.
- Diffuse scleritis or nodular scleritis
- Treatment of infectious scleritis: Systemic treatment with or without topical antimicrobial therapy always is required. Differentiating infectious scleritis from noninfectious scleritis is important because corticosteroid therapy and immunosuppressive therapy (often used in noninfectious autoimmune scleritis) are contraindicated in active infections.
Surgical Care
- Tectonic surgical procedures rarely may be required to preserve the integrity of the globe.
- Scleral grafts are fresh sclera or glycerin-preserved sclera that is available through eye banks. Grafts may be performed in cases of pending perforation during the time before the effects of systemic immunosuppressive agents manifest.[18]
- Corneal tissue may be used for associated corneal disease.
Consultations
- Consultation with a rheumatologist or internal medicine specialist for associated disease
- Consultation with a hematologist, oncologist, or internal medicine specialist for immunosuppressive therapy
Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis associated with rheumatoid arthritis and with other systemic immune-mediated diseases. Ophthalmology. Jul 1994;101(7):1281-6; discussion 1287-8. [Medline].
Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis associated with systemic vasculitic diseases. Ophthalmology. Apr 1995;102(4):687-92. [Medline].
French DD, Margo CE. Postmarketing surveillance rates of uveitis and scleritis with bisphosphonates among a national veteran cohort. Retina. Jun 2008;28(6):889-93. [Medline].
[Guideline] Zimmerman RD, Seidenwurm DJ, Davis PC, et al. ACR Appropriateness Criteria. Orbits, vision, and visual loss. National Guideline Clearinghouse. 2006.
Nieuwenhuizen J, Watson PG, Emmanouilidis-van der Spek K, Keunen JE, Jager MJ. The value of combining anterior segment fluorescein angiography with indocyanine green angiography in scleral inflammation. Ophthalmology. Aug 2003;110(8):1653-66. [Medline].
Hakin KN, Ham J, Lightman SL. Use of orbital floor steroids in the management of patients with uniocular non-necrotising scleritis. Br J Ophthalmol. Jun 1991;75(6):337-9. [Medline].
Galor A, Jabs DA, Leder HA, Kedhar SR, Dunn JP, Peters GB 3rd. Comparison of antimetabolite drugs as corticosteroid-sparing therapy for noninfectious ocular inflammation. Ophthalmology. Oct 2008;115(10):1826-32. [Medline].
Sobrin L, Christen W, Foster CS. Mycophenolate mofetil after methotrexate failure or intolerance in the treatment of scleritis and uveitis. Ophthalmology. Aug 2008;115(8):1416-21, 1421.e1. [Medline].
Wakefield D, McCluskey P. Cyclosporin therapy for severe scleritis. Br J Ophthalmol. Sep 1989;73(9):743-6. [Medline].
Cazabon S, Over K, Butcher J. The successful use of infliximab in resistant relapsing polychondritis and associated scleritis. Eye (Lond). Feb 2005;19(2):222-4. [Medline].
Huynh N, Cervantes-Castaneda RA, Bhat P, Gallagher MJ, Foster CS. Biologic response modifier therapy for psoriatic ocular inflammatory disease. Ocul Immunol Inflamm. May-Jun 2008;16(3):89-93. [Medline].
Murphy CC, Ayliffe WH, Booth A, Makanjuola D, Andrews PA, Jayne D. Tumor necrosis factor alpha blockade with infliximab for refractory uveitis and scleritis. Ophthalmology. Feb 2004;111(2):352-6. [Medline].
Sobrin L, Kim EC, Christen W, Papadaki T, Letko E, Foster CS. Infliximab therapy for the treatment of refractory ocular inflammatory disease. Arch Ophthalmol. Jul 2007;125(7):895-900. [Medline].
Ahmadi-Simab K, Lamprecht P, Nolle B, Ai M, Gross WL. Successful treatment of refractory anterior scleritis in primary Sjogren's syndrome with rituximab. Ann Rheum Dis. Jul 2005;64(7):1087-8. [Medline].
Cheung CM, Murray PI, Savage CO. Successful treatment of Wegener's granulomatosis associated scleritis with rituximab. Br J Ophthalmol. Nov 2005;89(11):1542. [Medline].
Papaliodis GN, Chu D, Foster CS. Treatment of ocular inflammatory disorders with daclizumab. Ophthalmology. Apr 2003;110(4):786-9. [Medline].
Onal S, Kazokoglu H, Koc A, Yavuz S. Rituximab for remission induction in a patient with relapsing necrotizing scleritis associated with limited Wegener´s granulomatosis. Ocul Immunol Inflamm. Sep-Oct 2008;16(5):230-232.
Sainz de la Maza M, Tauber J, Foster CS. Scleral grafting for necrotizing scleritis. Ophthalmology. Mar 1989;96(3):306-10. [Medline].
Fong LP, Sainz de la Maza M, Rice BA, Kupferman AE, Foster CS. Immunopathology of scleritis. Ophthalmology. Apr 1991;98(4):472-9. [Medline].
Sainz de la Maza M, Jabbur NS, Foster CS. An analysis of therapeutic decision for scleritis. Ophthalmology. Sep 1993;100(9):1372-6. [Medline].
Sainz de la Maza M, Jabbur NS, Foster CS. Severity of scleritis and episcleritis. Ophthalmology. Feb 1994;101(2):389-96. [Medline].
Tuft SJ, Watson PG. Progression of scleral disease. Ophthalmology. Apr 1991;98(4):467-71. [Medline].
Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol. Mar 1976;60(3):163-91. [Medline].

