Scleritis. Is it or is it not. That is the question.

Is it or isn't it?  The diagnosis of scleritis is a serious one and can result in very severe damage to the eye.  Scleritis occurs in patients who typically have other autoimmune disease.  When a patient with persistently red eyes and an autoimmune disease presents to their physician, the possibility of scleritis should be considered.

Scleritis is not simply redness of the eyes, but also inflammation of the sclera, the episcleral vessels. It is very difficult to distinguish from garden variety conjunctivitis in some cases.  The most important diagnostic feature is a thickening of either the anterior or posterior sclera. Without aggressive treatment, and sometimes even with such treatment, scleritis can result is severe damage to the eye as shown in this photo series showing scleritis over a several year period.

Notice how it looks like a bad case of pink eye in 2003. By 2010, more than pink eye. 

Emedicine Overview of Scleritis

One of our local rheumatologists recently referred a patient for possible scleritis.  This was based upon the combination of a systemic auto-immune condition (urticara vasculitis) and symptoms of persistently painful red eyes.  We were able to demonstrate with confidence that the patient did not have actual swelling of the sclera by using a novel technique.

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Initially, we carried out B scan ultrasound to look for signs of thickening of the scleral walls or a "flat tire" sign.  This is helpful in identifying posterior scleritis.  For anterior sclera, it is quite difficult to know if there is thickening.  To make it more difficult, scleritis can occur in different variants such as "nodular" or "diffuse" making some areas of the sclera more swollen than others.

B Scan showing scleritis. Note thickening and distortion.
Photo: Digital Journal of Opthalmology

Ultrasound Biomicroscopy (UBM) is a new imaging modality for the anterior segment which should be helpful to identify swelling of the anterior sclera.  Unfortunately, this requires contact with the eye which can be very uncomfortable.

This happened with our patient.  Another imaging modality turned out to be more useful.  Using a spectral domain OCT with a near focus lens attachment, we were able to non invasively and without contact image the anterior segment in beautiful detail.  The images showed quite clearly that there was a smooth non thickened, non lumpy or nodular sclera in the anterior segement of both eyes.

A External appearance. BVector for anterior segment OCT. C Image of anterior sclera showing thickening of the sclera with pockets of fluid.  

Great Article on Use of Anterior Segment OCT for Scleritis

Based on our findings, we recommended that the patient stabilize his systemic immunosuppressants and initiate therapy with two topical medications.  One, Durezol TID and the second Cyclosporine drops.  This powerful combination should reduce superficial inflammation quickly and bring relief to the patient.  We will update you on his progress.  Use of Restasis for this condition is based on: need for improving conjunctival mucin layer as well as knowledge that scleral and epi-scleral inflammation may respond directly to immunomodulation with cyclosporine.  Let's cross our fingers for a quick response.

Is there any evidence that CSA is of benefit for scleritis? 

First Report of Scleritis treatment with Cyclosporine drops

Without knowing for sure whether there is thickening of the sclera, we might be tempted to treat him for scleritis, just in case.  The full treatment however requires high doses of powerful systemic immunosuppressant drugs for a prolonged period.  Many side effects can occur in both eye and the rest of the body.  These include cataracts and glaucoma for the eye and immune system failure, aseptic necrosis of the hip, and GI bleeding as systemic effects.  Instead, we chose to treat with topical agents that have fewer side effects than oral medication.  Although use of Restasis for scleritis sounds like a novel idea, it has been reported before.

By confidently determining the scleral thickness, we gain diagnostic precision and a valuable baseline from which to measure should we think he is evolving to scleritis.

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