• Noninfectious Uveitis: Basic Concepts of Immunosuppression

    Scleritis

    Uveitis is one of the leading causes of vision loss, accounting for 10 to 20 percent of blindness in the United States.

    The majority (close to 90 percent) of uveitis cases are noninfectious. When they become chronic, the management can be challenging and often requires immunomodulatory therapy (IMT). Given the limited access to uveitis specialists, the diagnosis and management of these patients very often relies on the comprehensive ophthalmologist or other subspecialists.

    Recent studies like the Multicenter Uveitis Steroid Treatment (MUST) trial and follow-up study, which compared local therapy (dexamethasone implant) versus systemic therapy (corticosteroids and immunosuppression), showed no increased risk of systemic side effects after seven years of follow up, except for increased use of antibiotics for infections in the systemic group. This means that when used correctly, systemic immunosuppression can be safe and prevent vision loss.

    Here are the basic concepts for the diagnosis and treatment of noninfectious uveitis:

    1. Rule out infectious causes: Before starting any type of immunosuppression, possible infectious etiologies should be ruled out. All patients should be tested for syphilis. Other infectious etiologies like herpetic disease should be excluded based on clinical findings (e.g., retinitis), patient medical or travel history. IMT should be considered only when infectious etiologies or possible masquerade syndromes (e.g., lymphoma) are excluded.
    1. Recognize diagnoses that need early immunosuppression: Recommendations from an expert panel in 2000 suggested starting IMT from the onset for certain diseases that have poor prognosis when untreated. These conditions include Behcet’s disease with posterior involvement, mucous membrane pemphigoid and scleritis associated with systemic vasculitis (granulomatosis with polyangiitis or polyarteritis nodosa). Serpiginous choroidopathy appears to benefit from early IMT but is too infrequent for recommendations to be definitive.
    1. How to start treatment: Oral corticosteroids are usually the first line of treatment and beneficial for most autoimmune diseases, but their use is limited due to multiple side effects. Starting dose is typically 1mg/kg up to 60 mg of prednisone, but if a patient fails to respond to oral corticosteroids, IMT should be added. Also, in patients who are unable to taper oral corticosteroids to a safe dose (chronic dose of prednisone ≤7.5 mg daily), IMT should be strongly considered.
    1. Monitoring: Once IMT is initiated, patients should have regular blood work to monitor for possible medication toxicities. Checking for white blood cell counts and liver/kidney function will almost always be part of the follow-up, done every four to 12 weeks depending on dose adjustment and physician preference. 
    1. Refer early: If you don’t feel comfortable managing IMT, are unable to bring the patient to a safe dose of corticosteroids or feel like it’s getting out of your hands, refer patients to a uveitis specialist. If one is not available, consult a rheumatologist to assist with the treatment. It is completely acceptable to recognize our limitations for the benefit of the patient, which might result in better patient care and visual outcomes.

    Further resources

    Focal Points 2012 Module: Biologic Response Modifiers in Uveitis

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    Stephanie Llop Santiago, MDAbout the author: Stephanie Llop Santiago, MD, is an ophthalmologist in New York and is affiliated with multiple hospitals, including Mount Sinai Beth Israel Hospital and Mount Sinai Hospital. She received her medical degree from the University of Puerto Rico School of Medicine.