Pseudoexfoliation (PXE) syndrome is an age-related disease that can occur in various parts of the body, but which mainly impacts the eye, increasing risk of glaucoma. Here, ophthalmologist Yvonne Ou, M.D. provides an overview of PXE, including what it is, who’s most at risk, what treatment options are available, and how it can be prevented.*

What is PXE and pseudoexfoliation glaucoma?

PXE syndrome happens when microscopic flakes of protein fibers, as well as pigment, build up primarily in the front part of the eye. This happens, for example, on the lens and in the drainage structures where excess fluid exits the eye. When the eye’s drainage system is blocked, eye pressure can rise leading to pseudoexfoliation glaucoma.

While protein exfoliation can happen in other organs, such as the heart and lungs, it appears to only be problematic when it occurs in the eye.

Are you at risk for PXE?

There are a handful of risk factors for PXE, including:

• Being 50 years of age or older
• Being female
• Being of Scandinavian descent
• Carrying an LOXL1 gene variant

Ou says PXE is generally rare in African Americans and Eskimos, but can be found in various parts of Europe, the Middle East, South America, and South Africa, and is “likely found in all populations.”

Is glaucoma likely with PXE syndrome?

About 40%-50% of people with PXE syndrome go on to develop pseudoexfoliative glaucoma. 

PXE syndrome can be subtle and get overlooked, as it has no symptoms and requires an eye exam to diagnose. “A careful examination by an ophthalmologist, including a detailed look at the drainage angle and a dilated eye exam, will help to determine the diagnosis,” says Ou. 

Once identified, people with PXE must be monitored for glaucoma development.

PXE features and treatments

Compared to primary open-angle glaucoma (POAG), eye pressure in pseudoexfoliation glaucoma can be “quite high” and more difficult to manage. There is also often greater visual field loss and optic nerve damage in this type of glaucoma than in POAG. 

Ou explains that pseudoexfoliation glaucoma can sometimes lead to acute angle-closure glaucoma (a medical emergency) if exfoliative materials deposit on the structures that support the eye lens. If these structures weaken, the lens can move forward and narrow the angle between the iris and cornea, impeding fluid drainage and raising eye pressure.

According to Ou, laser iridotomy is sometimes recommended to help prevent acute angle-closure, and in some cases surgeries such as trabeculectomy are necessary to control eye pressure long-term.

Cataract surgery and PXE syndrome

Because PXE syndrome can cause the eye lens to move and pressure to increase, complications can arise during, and even after, cataract surgery. Surgeons are aware of this, Ou says, and are appropriately cautious.

Cataract patients are also advised not to wait too long to undergo surgery, since the cataract can become denser over time, making surgery more difficult.

“If you have pseudoexfoliation syndrome or pseudoexfoliation glaucoma, it is important to have a frank discussion with your ophthalmologist prior to cataract surgery so that you fully understand the risks,” Ou advises.

Can PXE be prevented?

Researchers are working to understand how the environment can trigger PXE. Until then, Ou says to take the same precautions as for any age-related eye disease, specifically:

• Get a baseline eye exam at age 40, with follow-up exams:
  • every 2-4 years until age 54, 
  • every 1-3 years from ages 55-64, and 
  • every 1-2 years from age 65 on, and 
• Wear sunglasses.

People who already have PXE syndrome need to maintain close follow-up with their ophthalmologist to monitor for glaucoma development and progression.

*Ou, Y. (2021, July 14). Pseudoexfoliation (PXE) Syndrome and Pseudoexfoliation Glaucoma. BrightFocus Foundation. https://www.brightfocus.org/glaucoma/article/pseudoexfoliation-pxe-syndrome-and-pseudoexfoliation-glaucoma