Peripheral Ulcerative Keratitis
(Marginal Keratolysis; Peripheral Rheumatoid Ulceration)
Peripheral ulcerative keratitis is a serious eye disorder that involves inflammation and ulceration of the cornea (the clear layer in front of the iris and pupil) that often occurs in people who have connective tissue disorders such as rheumatoid arthritis.
Peripheral ulcerative keratitis is probably caused by an autoimmune reaction. In an autoimmune disorder, antibodies or cells produced by the body attack the body’s own tissues. Many autoimmune disorders affect connective tissue in a variety of organs. Connective tissue is the structural tissue that gives strength to joints, tendons, ligaments, and blood vessels. The connective tissue disorders that occur with peripheral ulcerative keratitis include rheumatoid arthritis, granulomatosis with polyangiitis, and relapsing polychondritis.
Peripheral ulcerative keratitis also may be caused by an infection.
The diagnosis of peripheral ulcerative keratitis is suspected when the doctor sees the affected cornea in a person who also has a severe and/or long-standing connective tissue disease.
If doctors suspect an infection, such as bacteria, fungi, or herpes simplex virus, is the cause of peripheral ulcerative keratitis, they scrape the ulcer and eyelid margins for a sample. The sample is then grown in a laboratory (cultured) to identify the organism.
Without treatment, about 40% of people who have an autoimmune connective tissue disease and peripheral ulcerative keratitis die (mostly due to a heart attack) within 10 years of developing peripheral ulcerative keratitis. With treatment, about 8% of people die within 10 years. The eye problem itself is not the cause of death, it is the underlying autoimmune disorder's effect on the whole body.
To treat peripheral ulcerative keratitis, doctors give drugs that suppress the immune system, such as methotrexate or cyclophosphamide taken by mouth or by vein (intravenously).
Doctors may also fill the ulcer with a special material (called tissue adhesive) and then cover the area with special contact lenses to control inflammation. Alternatively, doctors may surgically repair the cornea with a partial thickness corneal transplant.