Scleritis Treatment & Management

Updated: May 09, 2017
  • Author: Manolette R Roque, MD, MBA, FPAO; Chief Editor: Andrew A Dahl, MD, FACS  more...
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Treatment

Medical Care

Treatment of scleritis almost always requires systemic therapy. Patients with an associated disease, such as rosacea, gout, atopy, or infection, need disease-specific treatment. Systemic therapy complements aggressive topical corticosteroid therapy, generally with difluprednate, prednisolone or loteprednol etabonate, thereby reducing the dose requirements for both topical and oral modalities. This combination therapy mitigates, to some extent, the inherent risks of oral steroids, including hypertension and diabetes, among many others; the risks of GI and renal morbidity caused by oral nonsteroidal anti-inflammatory drugs (NSAIDs); and the risks of elevated IOP and cataractogenesis due to topical steroids.

Treatment of noninfectious scleritis: Systemic NSAIDs, corticosteroids, or immunomodulatory drugs are indicated. [10] Topical therapy is routinely insufficient. This treatment must be individualized for the severity of the scleritis, response to treatment, adverse effects, and presence of associated disease.

Dosing information is as follows:

  • Methotrexate 7.5-25 mg PO/IM once a week
  • Azathioprine 1-2.5 mg/kg/day PO
  • Cyclophosphamide 1.5-3 mg/kg/day PO
  • Mycophenolate mofetil 1 g PO bid
  • Infliximab 3-5 mg/kg IV q6-8wk

Diffuse scleritis or nodular scleritis

The initial therapy consists of an NSAID; in case of therapeutic failure, 2 additional successive different NSAIDs should be tried following 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, particularly when a comorbid infectious etiology such as toxoplasmosis or syphilis cannot be completely ruled out. [11]

In case of therapeutic failure of systemic corticosteroids, immunosuppressive drugs should be added or substituted. Methotrexate (MTX) can be the first choice, but azathioprine, mycophenolate mofetil, cyclophosphamide, or cyclosporine may also be helpful. [12, 13, 14] Cyclophosphamide should be the first choice in treating patients with associated potentially lethal vasculitic diseases, such as granulomatosis with polyangiitis or polyarteritis nodosa.

In case of therapeutic failure, biologic response modifiers, such as infliximab or adalimumab, may be effective. [15, 16, 17, 1, 6] Other alternatives include golimumab, certolizumab, tocilizumab, and rituximab, although further investigation is warranted. [18, 19, 20]

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 granulomatosis with polyangiitis or polyarteritis nodosa.

In case of therapeutic failure, biologic response modifiers, such as infliximab or adalimumab, may be effective. Other alternatives are golimumab, certolizumab, tocilizumab, and rituximab, although their efficacy awaits further study. [20, 21]

Periocular steroid injections should not be applied in cases of necrotizing scleritis or peripheral ulcerative keratitis but could be very helpful in diffuse or nodular scleritis as an adjunctive therapy. 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.

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.

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Surgical Care

Tectonic surgical procedures rarely may be required to preserve the integrity of the globe.

Scleral grafts from fresh donor sclera or glycerin-preserved sclera are available through eye banks. Grafts may be performed in cases of pending perforation during the time before the effects of systemic immunosuppressive agents manifest.

Corneal tissue may be used for associated corneal disease.

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Consultations

See the list below:

  • Consultation with a rheumatologist or internal medicine specialist for associated disease
  • Consultation with a hematologist, oncologist, or internal medicine specialist for immunomodulatory therapy
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Precautions

Drug precautions are as follows:

  • Avoid methotrexate if creatinine clearance <10; contraindicated in chronic hepatic disease;, screen for hepatitis A, B, and C; give hepatitis vaccination; decrease or avoid alcohol intake
  • Azathioprine is associated with an increased risk of pancreatitis; decrease dose 50% if creatinine clearance <10
  • Decrease cyclophosphamide dose 25% if creatinine clearance <10; caution advised in patients with hepatic impairment
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