Uncovering a Significant Cause of Dry Eye

Published in OneWilmer - Spring 2020

Despite affecting more people than rheumatoid arthritis, Sjögren’s syndrome is considerably less well known. While 1.2 million individuals in the U.S. have rheumatoid arthritis, 1.4 million people have a diagnosis of Sjögren’s syndrome — 90% of which are women. The two conditions are often compared because both are autoimmune diseases.

Like all autoimmune disorders, Sjögren’s syndrome causes the immune system to attack the body. The most common symptoms people with the condition experience are dry mouth and dry eyes. The latter symptom is what brings them into the care of Esen Akpek, M.D., director of the Ocular Surface Disease and Dry Eye Clinic at Wilmer.

Akpek has spent more than a decade examining the prevalence and consequences of the disease. Her interest stemmed directly from her experience with patients. She noticed a lot of her patients presenting with dry eye had fatigue, dry mouth and joint pains. “I started working them up — sending them to rheumatology, internal medicine, ENT,” says Akpek. They found that many of these patients had Sjögren’s syndrome.

That eventually led to a paper that predicts the incidence of Sjögren’s syndrome among patients with dry eye is much higher than previously thought. “What was known is 90-something percent of patients with Sjögren’s have dry eye. But what percentage of dry eye patients have Sjögren’s was not known,” says Akpek. “In 2009, we showed that 1 in 10 do — that’s a very high percentage.” Akpek believes the actual number of people with Sjögren’s syndrome is closer to 4 million.

Her work also demonstrated that there is a 10-year lag time in the diagnosis of Sjögren’s syndrome, which is significant because of the disease’s potential complications — from systemic ones such as lymphoma or hepatitis to localized ones such as corneal scarring and blindness.

“Of all the autoimmune diseases, patients with Sjögren’s syndrome are the most likely to develop lymphoma over their lifetime,” says Akpek. “It’s a very important disease.”

It is also a disease that requires teamwork. As associate director of the Johns Hopkins Jerome L. Greene Sjögren’s Syndrome Center, she partners with doctors throughout the hospital — particularly rheumatologists— to treat patients.

Generally, ophthalmologists do not treat patients with systemic medications, but rheumatologists do. “Sjögren’s is a multisystemic, multi-organ system disorder. No one person can handle the entire patient. You need experts in different kinds of fields,” says Akpek. “Collaboration is necessary.”