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Woman presents with 3-week history of redness in left eye
The patient was diagnosed with Wegener’s granulomatosis 20 years before presentation.
A 62-year-old woman presented to the New England Eye Center for evaluation of left eye redness present for the past 3 weeks. She denied any significant discomfort but felt that her vision was mildly decreased from baseline.
 Isabel M. Balderas |  Tom Hsu | ||
She had been seen by an optometrist 10 days before presentation and was instructed to use Tobradex (tobramycin-dexamethasone, Alcon) every 3 hours in the affected eye. The redness subsided with Tobradex, but it quickly returned once the medication was discontinued.
A review of systems was remarkable for an episode of sinusitis that began around the same time as her eye redness. This was treated with oral antibiotics by her primary care provider and was improving. Her medical history included Wegener’s granulomatosis diagnosed by lung biopsy approximately 20 years prior. She was followed by a rheumatologist and had no exacerbations for nearly 15 years.
Examination
Examination revealed a visual acuity of 20/30 in the right eye and 20/70 in the affected left eye. The patient’s visual acuity improved in the left eye to 20/40 with pinhole. Pupil exam was normal. IOP by tonometry was 14 mm Hg in both eyes. The patient had sectoral conjunctival injection of her left eye and a corneal lesion (Figure 1). The anterior chambers in both eyes were free of cell and flare. Lenses each had 1+ nuclear sclerosis. Dilated fundus examination revealed no abnormalities.
 Color photo of the left eye shows sectoral conjunctival injection with a superonasal peripheral corneal lesion. Image: Raizman MB, Swanic M |
What is your diagnosis?
Eye redness
The patient was diagnosed with peripheral ulcerative keratitis, thought to be secondary to her Wegener’s granulomatosis. Tobradex was discontinued, and she was started on oral prednisone 40 mg daily.
Upon follow-up after 1 week, there was noted to be improvement in her vision and ocular redness, and the prednisone was tapered to 30 mg daily. However, repeat examination 1 week later revealed a mild increase in redness in her left eye and new infiltrates in her right eye. At this point, rheumatology recommended treatment with cyclophosphamide, but the patient refused due to prior side effects. Instead, therapy with azathioprine was initiated, and the prednisone was increased. One week follow-up revealed resolution of redness and return of visual acuity to 20/25, her baseline vision in the left eye. She presently continues on azathioprine and prednisone.
Discussion
Peripheral ulcerative keratitis is an autoimmune disease most commonly associated with rheumatoid arthritis. It is also seen in Wegener’s granulomatosis, systemic lupus erythematosus, relapsing polychondritis, ulcerative colitis and polyarteritis nodosa. Biopsy of conjunctival tissue adjacent to the ulceration has revealed immune-mediated veno-occlusive disease.
Peripheral ulcerative keratitis can be the first sign of an underlying systemic condition and has been reported to frequently be the first sign of Wegener’s granulomatosis. On the other hand, it is typically a late finding in patients with rheumatoid arthritis. McKibben and colleagues reported peripheral ulcerative keratitis to be a late finding that often occurs between 12 and 35 years after initial diagnosis of rheumatoid arthritis. Foster and colleagues reported a high rate of mortality among patients with rheumatoid arthritis who did not receive aggressive systemic treatment. This study supports the general approach in the management of peripheral ulcerative keratitis that includes aggressive systemic immune-modulating treatment including cytotoxic agents.
Management of peripheral ulcerative keratitis in patients with rheumatoid arthritis classically has been with methotrexate and systemic corticosteroids while that for Wegener’s granulomatosis has been with cyclophosphamide and systemic corticosteroids. A recent case report by Freidlin and colleagues showed the new biologic medication rituximab to be beneficial in a patient with Wegener’s granulomatosis and peripheral ulcerative keratitis refractory to corticosteroids and cyclophosphamide. Rituximab is a monoclonal antibody targeting the CD-20 molecule present on all B-cells before conversion into plasma cells. It was developed for treatment of lymphoproliferative disease, but it is yielding success in the treatment of immune-mediated disorders as well.
For more information:
Matthew Swanic, MD, and Michael B. Raizman, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.
- Edited by Isabel M. Balderas, MD, and Tom Hsu, MD. Drs. Balderas and Hsu can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Balderas and Hsu have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.
References:
- Foster CS, Forstot SL, Wilson LA. Mortality rate in rheumatoid arthritis patients developing necrotizing scleritis or peripheral ulcerative keratitis. Effects of systemic immunosuppression. Ophthalmology. 1984;91(10):1253-1263.
- Freidlin J, Wong I, Acharya N. Rituximab treatment for peripheral ulcerative keratitis associated with Wegener’s granulomatosis. Br J Ophthalmol. 2007;91(10):1414.
- Ladas J, Mondino B. Systemic disorders associated with peripheral corneal ulceration. Curr Opin Ophthalmol. 2000;11(6):468-471.
- McKibbin M, Isaacs JD, Morrell AJ. Incidence of corneal melting in association with systemic disease in the Yorkshire Region, 1995-1997. Br J Ophthalmol. 1999;83(8):941-943.