BY WILLIAM L. MILLER, OD, MS. PHD, FAAO
Clinical conditions that cause conjunctival hyperemia can range from benign to the more serious, with high rates of ocular morbidity if left untreated. Scleritis is an uncommon condition that fits the latter and can lead to severe vision loss and, in rare cases, loss of the eye. In at least one study in Northern California, the annual prevalence of scleritis was reported to be 5.2 per 100,000 persons (Honik et al, 2013). It affects mostly females in the fourth to sixth decade of life.
Scleritis is often associated with systemic disease (25% to 50%), more frequently than is episcleritis. The most commonly associated systemic diseases include rheumatoid arthritis, Wegener’s granulomatosis, relapsing polychondritis, systemic lupus erythematosus, inflammatory bowel disease, and seronegative spondyloarthropathies. Of these, rheumatoid arthritis is the most frequently associated (10.3% to 18.6%) (Akpek et al, 2004; Tuft and Watson, 1991; Sainz de la Maza, 1991).
For these reasons, patients who have scleritis should undergo the following blood tests: anti-neutrophil cytoplasmic antibody (ANCA), fluorescent treponemal antibody (FTA), erythrocyte sedimentation rate (ESR), and rheumatoid factor. A chest x-ray should also be performed (Watson, 1980; Lin et al, 2008).
Signs and Symptoms
Patients will present with a red and painful eye. At first just an ache, it progresses to a boring pain that radiates to the scalp and periorbital area. Diffuse or sectoral redness can appear and will take on a deeper red appearance in contrast to the redness of episcleritis. Oftentimes, episcleritis will appear along with the scleritis. Application of 2.5% phenylephrine will help distinguish the episcleritis from the deeper scleritis hyperemia. The sclera will appear chemotic with biomicroscopy or anterior segment ocular coherence tomography.
Five subtypes of scleritis occur, all but one in the anterior sclera. The subtypes include anterior scleritis (diffuse, nodular, necrotizing with inflammation, necrotizing without inflammation) and posterior scleritis. Severity and number of episodes will cause thinning of the sclera, which will cause the sclera to appear blue. Necrotizing forms of scleritis have greater chances of ocular and systemic complications, with up to 30% demonstrating a loss of visual acuity (Jabs et al, 2000).
Treatment and management of scleritis patients requires coordinated care between optometry, ophthalmology, internal medicine, and rheumatology. Scleritis patients are typically unresponsive to topical nonsteroidal anti-inflammatory (NSAID) and corticosteroid medications. However, 33% to 66% of patients experience symptom resolution with oral NSAIDs (Rosenbaum and Robertson, 1993; Jabs et al, 2000), which represent the first line of therapy for non-necrotizing scleritis (Spiegel et al, 2005). Oral corticosteroids remain the first line of treatment in necrotizing inflammation. Corticosteroids can also be used in initial cases of scleritis except in those caused by infectious agents. The most common infectious agent is herpes zoster, and antivirals such as acyclovir and valacyclovir are the first-line therapies.
Up to 25% of patients who have scleritis require immunosuppressive medications (Jabs et al, 2000), such as methotrexate, cyclosporine, azathioprine, mycophenolate, and mofetil. Biologic agents such as infliximab, daclizumab, adalimumab, and rituximab have also been used (Murphy et al, 2004). A recent panel of experts indicated that infliximab and adalimumab serve as second-line immunomodulatory agents for scleritis (Levy-Clarke et al, 2014). CLS
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Dr. Miller is an associate dean for academic affairs and professor at the Rosenberg School of Optometry, University of the Incarnate Word. He is a consultant or advisor to Alcon and Oasis Medical and has received research funding from CooperVision, Contamac, and SynergEyes and lecture or authorship honoraria from Alcon. You can reach him at firstname.lastname@example.org.