Treatment of scleritis
- Reza Dana, MD, MPH, MSc
Reza Dana, MD, MPH, MSc
- Claes Dohlman Professor of Ophthalmology
- Harvard Medical School
Scleritis is a painful, destructive, and potentially blinding disorder that may also involve the cornea, adjacent episclera, and underlying uveal tract. Up to 50 percent of patients with scleritis have an underlying systemic illness, most often a rheumatic disease.
This topic will review the treatment of scleritis. The clinical manifestations and diagnosis of scleritis are presented separately. (See "Clinical manifestations and diagnosis of scleritis".)
Treatment of scleritis always requires systemic therapy with nonsteroidal antiinflammatory drugs (NSAIDs), glucocorticoids, or other immunosuppressive drugs. In one study, 67 percent of patients required either high-dose glucocorticoids or the combination of high-dose glucocorticoids and another immunosuppressive agent to control the disease . In some patients, particularly those with peripheral ulcerative keratitis or scleromalacia perforans, surgical intervention is required to preserve vision or prevent globe rupture.
General principles — Treatment must be individualized according to the severity of the patient's disease. As an example, patients with either necrotizing anterior scleritis or posterior scleritis require more intensive therapy than those who present with non-necrotizing disease of the anterior . The presence of a systemic inflammatory illness may also dictate an intensive course of therapy, even if the particular subtype of scleritis would normally call for a more benign treatment approach. (See "Clinical manifestations and diagnosis of scleritis", section on 'Scleritis subtypes'.)
It is absolutely essential that scleritis be managed by an ophthalmologist expert in the care of these patients and by a rheumatologist experienced in employing and managing the immunosuppressive therapies required to treat ocular inflammation.
- Jabs DA, Mudun A, Dunn JP, Marsh MJ. Episcleritis and scleritis: clinical features and treatment results. Am J Ophthalmol 2000; 130:469.
- McCluskey PJ, Watson PG, Lightman S, et al. Posterior scleritis: clinical features, systemic associations, and outcome in a large series of patients. Ophthalmology 1999; 106:2380.
- Tuft SJ, Watson PG. Progression of scleral disease. Ophthalmology 1991; 98:467.
- Thorne JE, Jabs DA, Qazi FA, et al. Mycophenolate mofetil therapy for inflammatory eye disease. Ophthalmology 2005; 112:1472.
- Sen HN, Suhler EB, Al-Khatib SQ, et al. Mycophenolate mofetil for the treatment of scleritis. Ophthalmology 2003; 110:1750.
- Jachens AW, Chu DS. Retrospective review of methotrexate therapy in the treatment of chronic, noninfectious, nonnecrotizing scleritis. Am J Ophthalmol 2008; 145:487.
- Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363:221.
- Specks U, Merkel PA, Seo P, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med 2013; 369:417.
- WGET Research Group. Design of the Wegener's Granulomatosis Etanercept Trial (WGET). Control Clin Trials 2002; 23:450.
- Hillenkamp J, Kersten A, Althaus C, Sundmacher R. [Cyclosporin A therapy in severe anterior scleritis. 5 severe courses without verification of associated systemic disease treated with cyclosporin A]. Ophthalmologe 2000; 97:863.
- McCarthy JM, Dubord PJ, Chalmers A, et al. Cyclosporine A for the treatment of necrotizing scleritis and corneal melting in patients with rheumatoid arthritis. J Rheumatol 1992; 19:1358.
- Ashok D, Ayliffe WH, Kiely PD. Necrotizing scleritis associated with rheumatoid arthritis: long-term remission with high-dose infliximab therapy. Rheumatology (Oxford) 2005; 44:950.
- El-Shabrawi Y, Hermann J. Anti-TNF alpha therapy in chronic necrotizing scleritis resistant to standard immunomodulatory therapy in a patient with Wegener's granulomatosis. Eye (Lond) 2005; 19:1017.
- Cazabon S, Over K, Butcher J. The successful use of infliximab in resistant relapsing polychondritis and associated scleritis. Eye (Lond) 2005; 19:222.
- Murphy CC, Ayliffe WH, Booth A, et al. Tumor necrosis factor alpha blockade with infliximab for refractory uveitis and scleritis. Ophthalmology 2004; 111:352.
- Doctor P, Sultan A, Syed S, et al. Infliximab for the treatment of refractory scleritis. Br J Ophthalmol 2010; 94:579.
- Wegener's Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener's granulomatosis. N Engl J Med 2005; 352:351.
- Pakrou N, Selva D, Leibovitch I. Wegener's granulomatosis: ophthalmic manifestations and management. Semin Arthritis Rheum 2006; 35:284.
- General principles
- Nonsteroidal antiinflammatory drugs
- - Tapering regimen
- - Pulse glucocorticoid therapy
- Immunosuppressive medications
- - Rituximab
- - Cyclophosphamide
- - Cyclosporine
- - Maintenance therapy
- Duration of therapy
- Resistant disease
- - Tumor necrosis factor inhibitors
- Role of surgery
- COURSE AND PROGNOSIS
- Disease complications
- - Corneal changes
- - Glaucoma
- - Cataracts
- - Posterior segment complications
- SUMMARY AND RECOMMENDATIONS