Title: Necrotizing scleritis
Author: Alexander Compton, MS IV
Photographer: James Gilman, CRA, FOPS
Image #1: Taken on April 27. This shows the superonasal quadrant of the patient’s sclera. There is scleral inflammation and a small ulcer in the center of the picture. There is a mucous glob overlying and a peripheral corneal infiltrate present. Notably, the uvea is not visible.
Image #2: Taken on May 2. This shows progression of the ulcer. There is ongoing inflammation present, and the ulcerous area has grown to at least triple its previous size, now measuring 5 by 7 mm and 3.6 by 3.8 mm. This very rapid progression over the course of just 5 days helps to confirm a diagnosis of necrotizing scleritis. Notably, there is still no uvea visible.
Image #3: Taken on June 20. This was taken after the patient began immunomodulating drug therapy but prior to any surgical intervention. Because of the immune therapy, the overall level of inflammation is down. However, by this point, the disease had progressed significantly. This picture (again with patient in down gaze in order to visualize the superonasal quadrant) demonstrates severe scleral thinning with loss of the overlying conjunctiva. The necrotic area measures 12 by 9 mm with a mucous glob overlying some of this area. The result is that the underlying uvea can be seen (the dark areas in the 10-12 o’clock region). The red area extending from the center of the photo to the 9 o’clock area is likely an early stage of uveal show. Though difficult to see near the bottom of this patient, the cornea had become very thin in the superonasal quadrant near the limbus.
Keywords/Main Subjects: Necrotizing scleritis, sclera, inflammation, scleral thinning, scleral ulcer, uveal show
Diagnosis: Necrotizing scleritis secondary to granulomatosis with polyangiitis (Wegener’s)
Description of Image:
Anterior scleritis is inflammation of the sclera anterior to the insertion of the recti muscles. It is associated with systemic immune or infectious disease in approximately 25% of cases. The characteristics that distinguish it from episcleritis are that 1) episcleritis tends to be more localized rather than diffuse, 2) a patient with episcleritis does not usually present with severe pain, 3) episcleritis is rarely associated with a systemic disease, and 4) since episcleritis involves more superficial layers, the blood vessels will blanch with use of 2.5-10% phenylephrine.
Necrotizing scleritis, a specific type of anterior scleritis, is rare but is the most severe form. It is characterized by rapid progression with infarction, necrosis, and thinning of the sclera and can lead to exposure of the underlying choroid. The prognosis in these patients is poor with high incidence of visual loss and increased rates of mortality.
This patient reported acute onset, non-traumatic redness and pain, potentially subsequent to prolonged contact use and occupational dust exposure. Patient was being followed closely while being treated with steroids for a few months without significant improvement. After the patient failed to follow-up for 2 months, he returned to clinic as pictured in image #1. At this point, an infectious cause was suspected; steroids were stopped, and antibiotic drops were initiated.
Five days later, the patient returned as pictured in image #2. Antiviral medication and oral steroids were added to the treatment regimen. President continued to be followed and eventually progressed to image #3 prior to receiving surgical intervention.
Though initial rheumatology testing came back negative, further lab testing came back positive for high PR-3 (serine protease 3 IgG) and p-ANCA thus increasing the likelihood that this case of necrotizing scleritis was indeed secondary to a systemic illness, specifically granulomatosis with polyangiitis.
“Scleritis — EyeWiki.” Accessed September 21, 2018. http://eyewiki.aao.org/Scleritis.
Leitman, Mark W. Manual for Eye Examination and Diagnosis. Hoboken: John Wiley & Sons Inc, 2016. Print.
Faculty Approval by: Griffin Jardine, MD
Disclosure (Financial or other): none