Some people living with rheumatoid arthritis (RA) can develop eye complications due to the same inflammation that attacks their joints. Peripheral ulcerative keratitis (PUK) — also called corneal ulceration — is an eye disease that may lead to sudden loss of vision. PUK has been associated with many autoimmune disorders, but RA is the most common, accounting for 34 percent to 42 percent of cases.

What Is PUK?

The cornea is the clear, dome-shaped tissue at the front of the eyeball. It acts as a window to the eye. The sclera is the white, outer layer of the eyeball that protects the cornea and surrounds most of the eyeball. When the cornea is damaged, vision becomes distorted or unclear. PUK develops when the cornea thins, is destroyed (corneal melt), or develops a hole (perforation).

PUK is rare, even in people with RA. One study estimated that PUK affects 1.4 percent of people with RA. Newer RA treatments, specifically biologic therapies, may help prevent the development of PUK. PUK typically develops many years after RA diagnosis. In two case studies, PUK developed between 17 to 20 years after RA diagnosis.

Serious PUK complications can be prevented with a timely diagnosis, early detection of RA, and proper treatment.

Signs and Symptoms of PUK

Signs of PUK include:

• Bloodshot eyes
• Swelling of the cornea
• A crescent-shaped sore (ulceration) next to the cornea
• Scleritis (inflammation of the sclera)
• Uveitis (inflammation of the uvea, which sits behind the cornea and sclera)

People with PUK may experience eye pain, sensitivity to light (photophobia), tearing, blurry vision, and loss of vision. In about 50 percent of cases of PUK, the condition occurs in both eyes in later stages of RA.

“My eyes were very red and felt like they were going to pop out, and I had bolts of pain shooting through my head,” wrote one myRAteam member about their PUK. “My doctor gave me eye steroids, which helped a lot, but I still have problems with bright lights.”

Causes of PUK

The underlying cause of PUK remains unknown. However, researchers believe that several factors may cause the condition. Systemic (whole body) infections, eye infections, genetics, eye trauma, and autoimmune processes within cells may all play a role.

Some risk factors of PUK include:

• Sex — Females are more likely to have both PUK and RA.
• Having a long history of RA — PUK typically develops late in the disease course of RA. It usually indicates worsening of the autoimmune disease.
• Certain biomarkers — High levels of two biomarkers (rheumatoid factor and anti-cyclic citrullinated peptide antibodies) in the blood can indicate higher risk for PUK.

When To See a Doctor

Speak to your rheumatologist if you have risk factors for PUK or if you begin to experience new or worsening eye symptoms. They can refer you to an ophthalmologist — a doctor specializing in eye diseases — who can evaluate your risk and potentially screen you for PUK.

During the ophthalmic exam, they will assess your vision, intraocular pressure, pupils, and overall eye health. They will also assess corneal thinning and your degree of inflammation. If you do have PUK, they can help determine the best treatment options for your symptoms.

Treating PUK With RA

A combination of immunosuppressive therapies and RA treatments can treat PUK associated with RA. Therapies recommended for PUK may include:

• Eye lubrication to promote tissue healing
• Soft contact lenses to “bandage” the cornea
• Steroid eye drops to relieve short-term inflammation
• Antibiotic eye drops to reduce infections
• Oral steroids to reduce inflammation
• Immunosuppressants such as methotrexate or cyclosporine to prevent more tissue damage
• Biologic disease-modifying antirheumatic drugs to prevent more inflammation

Ocular surgery is required in severe cases of PUK, such as for people with a high risk for corneal perforation. The type of ocular surgery depends on the size of the ulceration in the cornea. Surgery for PUK may include transplantation of corneal or scleral tissue.

Talk With Others Who Understand

On myRAteam, the social network for people with rheumatoid arthritis, more than 188,000 members come together to ask questions, give advice, and share their stories with others who understand life with RA.

Are you living with PUK and rheumatoid arthritis? How have you managed your eye condition, and what has helped you feel better? Share your thoughts in the comments below, or start a conversation by posting on your Activities page.