Pain, anterior status help distinguish scleritis from episcleritis
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Understanding the difference between scleritis and episcleritis is critical to minimizing eye pain and, potentially, preserving a patient’s vision, according to a presenter at the 2021 Congress of Clinical Rheumatology-West.
Jason R. Kolfenbach, MD, associate professor of medicine and ophthalmology and director of the rheumatology fellowship program at the University of Colorado, Boulder, suggested that applying the vasoconstrictor phenylephrine is the easiest way to differentiate between episcleritis or scleritis.
“If you have [episcleritis], it will go away, because there are episcleral vessels that are inflamed,” Kolfenbach said. “If you apply phenylephrine to scleritis, that is a deeper redness of an avascular structure, and it won’t clear.”
Kolfenbach noted that 75% of episcleritis cases are idiopathic. This complication can occur in about 25% of connective tissue diseases and in 10% of atopy, rosacea or gout cases.
Episcleritis generally has mild pain and no impact on vision, according to Kolfenbach. “It is often unilateral, but it can be recurrent,” he said, noting that 70% of patients have recurring attacks. Associated keratitis has been reported in 15% of patients. “The important thing is that it often does not progress to scleritis.”
Treatment of episcleritis is often administered through ophthalmology and may involve topical steroids. “Sometimes systemic NSAIDs are helpful for pain relief,” Kolfenbach said.
Scleritis is generally “much more painful” than episcleritis, Kolfenbach added. “If you have the patient close their eyes and push on the globe itself, it is usually very painful, as well,” he said.
Scleritis is idiopathic half of the time. It is seen most commonly in rheumatoid arthritis and granulomatosis with polyangiitis (GPA) and less commonly in inflammatory bowel disease. “Scleritis is much more commonly associated with autoimmune disease,” he said.
Ninety-eight percent of scleritis cases are anterior, according to Kolfenbach. “This means you and I could see them when they walk into the room,” he said.
Some 40% of anterior cases are diffuse, while 44% are nodular and 14% are necrotizing.
Posterior cases, which occur at the back of the eye, are considerably more challenging to manage. “How would you be tipped off to that?” he said. “If a patient has deep, boring pain, particularly if they have RA or GPA, where we know scleritis can happen, it would be reasonable to get them into the ophthalmologist.”
Kolfenbach acknowledged that even he has missed this diagnosis on occasion, which is more reason for rheumatologists to keep it on the radar. “Speaking as somebody who should have known better and has seen patients in the eye clinic for 7 years, you can get burned on it,” he said.
Bilateral disease occurs in 50% to 60% of posterior scleritis cases, while half will also involve associated keratitis or uveitis. In addition, about half of cases may impact the cornea and, by association, the patient’s vision.
Regarding treatment of scleritis, NSAIDs may be used as first-line therapy but are often ineffective against more severe cases. “Usually, by the time they see us, they are on prednisone,” Kolfenbach said, noting that these cases can be treated like an RA flare, with a “quick burst and taper” of steroids over a 4 to 6-week period.
For patients who fail to respond to this approach, methotrexate and azathioprine are the next drugs in line. “If a patient has thinning of the sclera, it is not uncommon to think about a big gun like [cyclophosphamide],” Kolfenbach said. “This is not something we take lightly in terms of the risk of that medication, but if you have globe rupture, it is very hard to rehab or recover.”