Systemic Treatment

Topical therapy is usually not sufficient to treat scleritis; therefore, treatment is systemic. It can range from oral nonsteroidal anti-inflammatory drugs (NSAIDs) to systemic immunomodulatory agents. In individuals with mild-to-moderate noninfectious anterior scleritis, either diffuse or nodular, primary management consists of oral NSAIDs for a few weeks, in the absence of specific contraindications. Caution is advised because of the risk of gastroduodenal ulceration, which may be decreased by concomitant use of histamine-H2 receptor antagonists or proton-pump inhibitors. It is also important to monitor renal function closely.

Severe noninfectious cases, refractory to NSAIDs or with posterior or necrotizing disease, are managed with systemic corticosteroids, typically with an initial dose of 1 mg/kg/day of prednisone or equivalent, followed by a slow tapering regimen. Careful monitoring for side effects is highly recommended.

When scleral inflammation recurs or is refractory to systemic corticosteroids, especially in necrotizing scleritis, immunomodulatory drugs (mainly antimetabolites, such as methotrexate, mycophenolate mofetil, or azathioprine) are indicated either as corticosteroid-sparing agents or as adjuvants. Biologic immunomodulatory agents, such as tumor necrosis factor inhibitors (eg, infliximab, adalimumab, and rituximab [anti-CD20]), have also been successfully employed for refractory cases.

Intravenous pulse therapy with methylprednisolone (1 g daily for 3 days, followed by high-dose oral prednisone) can be initially used in the setting of severe necrotizing or nonnecrotizing scleritis. Alkylating agents, such as cyclophosphamide, are reserved for severe necrotizing involvement of the sclera and cornea, with impending risk of perforation. They may also be helpful for the control of underlying systemic diseases (eg, granulomatosis with polyangiitis). Scleritis associated with granulomatosis with polyangiitis (formerly called Wegener granulomatosis) and polyarteritis nodosa typically requires more aggressive therapy with rituximab or cyclophosphamide.

Infectious scleritis can be treated with systemic (and topical) antimicrobials and surgical debridement as needed. It is important to differentiate infectious scleritis from non-infectious scleritis, because unopposed corticosteroids or immunosuppressive agents can worsen scleritis in the presence of active infection. Microorganisms may be difficult to eradicate from the sclera, and long-term treatment may be necessary. Scleral patch grafting may be necessary, if there is severe thinning.

Individuals with nonnecrotizing noninfectious scleritis who cannot tolerate systemic therapy or have residual scleral inflammation may be candidates for subconjunctival injection of low-dose subconjunctival triamcinolone (0.5–4 mg). It is important for each case to weigh the risks of cataract and secondary ocular hypertension/glaucoma and, more remotely, of scleral melting and infection against the possible benefits of the injection. Underlying systemic inflammatory diseases should be under control and infectious etiologies must be ruled out before considering local injection of steroids.

Topical corticosteroids can be used to control associated iridocyclitis, present in nearly one-third of individuals with scleritis. This may prevent complications such as anterior/posterior synechiae, uveitic glaucoma, and cataract.

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• Sohn EH, Wang R, Read R, et al. Long-term, multicenter evaluation of subconjunctival injection of triamcinolone for non-necrotizing, noninfectious anterior scleritis. Ophthalmology. 2011;118(10):1932–1937.

Excerpted from BCSC 2020-2021 series: Section 9 - Uveitis and Ocular Inflammation. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.