Early intervention considered key in diagnosis and treatment of scleritis

Take-home message: Scleritis can indicate serious systemic disease and requires prompt diagnosis and treatment. Episcleritis also has certain treatment pearls.

By Vanessa Caceres; Reviewed by Vincent de Luise, MD, FACS

New Haven, CT-Scleritis requires an early and accurate diagnosis to help patients recover from this potentially sight- and life-threatening disease, said Vincent de Luise, MD, FACS.

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“We need to confront these cases confidently, diagnose them accurately, and provide prompt, correct intervention,” said de Luise, MD, FACS, assistant clinical professor of ophthalmology, Yale University School of Medicine, New Haven, CT.

Scleritis is a painful, progressive disease characterized by inflammation and edema in the sclera and deep episclera. Corneal and external disease specialists are more likely than general ophthalmologists to see scleritis regularly. Scleritis is more common in females. In 50% of cases, it is idiopathic.

“In the other 50%, there is an underlying systemic disease,” Dr. de Luise said.

The underlying disease could be an immune-mediated systemic disease, and the strongest association is with vasculitis. Associated diseases include rheumatoid arthritis, Wegener’s granulomatosis, polyarteritis nodosa, and herpes zoster.

In a quarter of the cases where there is underlying disease, the eye is the presenting sign.

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“In these cases, the eye is the barometer, and the initial sign of a potentially lethal disease,” Dr. de Luise said.

Scleritis is almost always anterior, and it is classified as diffuse, nodular, necrotizing with inflammation, or necrotizing without inflammation. Posterior scleritis is rare and can mimic a choroidal tumor.

Next: Making the distinction

Pain is a distinguishing factor between scleritis and episcleritis, Dr. de Luise said.

“If there is pain, consider scleritis; if it’s just itching, consider episcleritis,” he said.

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In an examination under natural light, patients with scleritis will have a bluish-red discoloration in the area with scleritis. Using neosynephrine 2.5%, vessels will blanch with episcleritis but not scleritis.

Ocular sequelae for scleritis include cataract, glaucoma, uveitis, and peripheral ulcerative keratitis.

Laboratory testing can help identify the type of scleritis, Dr. de Luise said.

Depending on the history, testing should include rheumatoid factor, anti-nuclear antibodies, human leukocyte antigen, anti-neutrophilic cycloplasmic antibodies, and tests for tuberculosis, Lyme disease, sarcoidosis, and gout. Imaging tests include CXR, B-scan, sinus films, and CT or MRI tests.

Next: Treatment strategies

To treat scleritis, Dr. de Luise has found oral nonsteroidal anti-inflammatory drugs (NSAIDs) to be of little value, and he advised moving immediately to oral steroids followed by oral cytotoxic immunosuppressives, assuming no systemic contraindications.

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Methotrexate, azathioprine, and cyclophosphamide, are cytotoxic immunosuppressives that have shown efficacy in scleritis, Dr. de Luise said.

Mycophenolate mofetil and cyclosporine have also been employed.

Dr. de Luise said to use periocular steroids with caution as they have the risks of scleral melt and perforation.

Oral tumor necrosis factor inhibitors (TNF-alpha inhibitors) and other biologics have also shown efficacy in patients with scleritis but should be prescribed in collaboration with a rheumatologist, as they have serious systemic side effects, Dr. de Luise said.

Of the biologics, infliximab, adalimumab, daclizumab, rituximab, and etanercept have been used in the management of recalcitrant cases of scleritis, with variable degrees of effectiveness.

For necrotizing scleritis, IV corticosteroids and IV methylprednisolone should be considered, Dr. de Luise advised.

If there is impending scleral perforation, Dr. de Luise said that tectonic scleral patch graft surgery is the recommended initial management as the diagnosis is being pursued and oral treatment begun.

Next: Episcleritis identification, treatment

In contrasting the presentation of scleritis with that of episcleritis, Dr. de Luise said the latter is a self-limited disease of the episclera.

“In episcleritis, the inflammation is superficial, and the sclera is minimally engorged, if at all,” he said.

Patients usually have a history of allergy. Episcleritis can be divided into two types: simple (diffuse) and nodular. Although episcleritis is usually idiopathic, it may be associated with trauma, gout, atopy, rheumatoid arthritis, lupus, or inflammatory bowel disease. There is rarely an anterior chamber reaction in episcleritis.

There is also a related form, called pingueculitis, where the episcleritis manifests as an inflamed pinguecula, he said.

Treatment for mild episcleritis includes sunglasses and artificial tears. If the patient has itching, the specialist can recommend topical anti-allergics. For discomfort, Dr. de Luise advised the use of topical NSAIDS. In recalcitrant cases, oral NSAIDs such as indomethacin 75 to 100 mg orally per day, and/or topical pulsed corticosteroids, can be used.

Vincent de Luise, MD, FACS

E-mail: vdeluisemd@gmail.com

This article was adapted from Dr. de Luise’s presentation during Cornea Subspecialty Day at the 2014 meeting of the American Academy of Ophthalmology. Dr. de Luise did not indicate any proprietary interest in the subject matter.