In this presentation to the SLE Workshop at HSS, Sergio Schwartzman, MD spoke about the various eye problems associated with lupus, including uveitis, conjunctival issues, discoid lupus, sjogren's syndrome, vasculitis of the retina, and scleritis, among other issues.
He began by explaining that before he delved into the way lupus relates to the eyes, he intended to provide a brief overview of the anatomy of the eye itself.
“The eye is a very elegant organ, and it’s an organ that’s very complicated and a lot more resilient than you think it is,” he began. “It can tolerate quite a bit of injury, surgery, stress, and disease, and still function normally.”
He went on to explain that the closest a physician can come to examining the nervous system as part of a physical examination is to take a close look at the patient’s eye. “Because when you look at the eye,” he explains, “you can actually see the optic nerve.”
Dr. Schwartzman pointed out that eye complications in those with lupus are not usually caused by lupus itself, but by medications commonly used to treat the other medical manifestations of lupus. Since lupus can affect the entire system, however, including each organ in the body, it is not surprising that it can, in some cases, affect all the different parts of the eye.
The pupil, Dr. Schwartzman explains, is the small black hole in the middle of the eye, which is actually an opening which opens and closes depending on how much light hits it. The iris – the colored part of the eye – causes the pupil to let in the appropriate amount of light. The sclera is the white part of the eye. The cornea – the outside part of the eye that protects the lens – is physically connected to the sclera. Some of the conditions that can affect the sclera can also affect the cornea.
Diagram of the anatomy of the eye.
[Image courtesy of the National Eye Institute (public domain)]
The retina – the back part of the eye – is where the light that comes through the pupil is projected and interpreted by the optic nerve. Just next to the retina is the choroid. Both the retina and the choroid are susceptible to inflammation. The inside of the eye is divided into two chambers: the anterior chamber and the posterior chamber. Both chambers contain fluid, and when there’s inflammation in the eye, a specialist can often see inflammatory cells in the fluid. Lining the eye is a thin tissue called the conjunctiva, which is often more familiar to patients than other parts of the eye due to its susceptibility to inflammation, a condition known as conjunctivitis.
The eye is an “immune privileged” organ, meaning that it is protected from our immune system much more so than any of our other organ systems except for the brain, ovaries, and testes. As humans evolved, the eye evolved in a way to minimize inflammation so the eye could protect itself, as it is a critical part of our survival.
But, autoimmune diseases like lupus and rheumatoid arthritis can be strong enough in terms of immune response that they can bypass the “immune protected” state. The eye has evolved in a way to minimize inflammation, but on the other hand it has to balance this with the ability to protect itself from infections. The way it accomplishes this is by balancing a protective effect called the “blood ocular barrier” with allowing the immune system some access to the eye.
The term “uveitis” refers to inflammation of the uvea, the vascular middle layer of the eye. Any part of the eye can become inflamed, including the eyelids, conjunctiva, sclera, iris, retina, choroids, and optic nerve.
This type of lupus affects the skin, leaving scars and rashes, and the skin of the eyelid is not protected from all the other potential skin manifestations of lupus. As a result, discoid lupus can also involve the eyelid. It may also cause the loss of eyelashes.
Something that is very common in lupus is “dry eye syndrome.” People who have dry eyes usually explain that it feels like they have sand in their eyes. It presents when there is inflammation in the lacrimal glands, which prevents the natural moistening of the eye to proceed normally.
Dry eyes are one of the most common autoimmune manifestations in the eye, regardless of whether they occur alone (a condition known as primary Sjogren's syndrome) or secondary to another disease such as lupus.
In lupus, the retina is a major area of involvement. People with lupus can get retinal vasculitis, which limits the blood supply to the retina, which can have significant effects on vision. The eye then attempts to repair itself, but when the retina tries to repair itself it forms new blood vessels which can form in areas of the eye that can impair vision. Blood vessels involved by vasculitis can bleed, which can also impact vision.
When vasculitis involves the small area near the center of the retina where the vision is sharpest, the macula, a large area of central vision can be lost.
To diagnose retinal vasculitis, a retinal angiogram is performed, involving the injection of dye into the retina. The doctor then takes a picture of the retina, showing the optic nerve and blood vessels that exit from it, and, if vasculitis is present, irregularities in the retina due to impairment of circulation. These irregularities cause that part of the retina to stop functioning leading to loss of vision in that area.
Furthermore, because vasculitis can affect performance of the nerves that cause the eye muscles to contract, those with lupus may experience involuntary movement of the eye, which can lead to double vision.
The white part of the eye, also known as the sclera, can become inflamed, causing a yellow discoloration to appear in a particular area. Scleritis, as this condition is known, is very unusual in those with lupus, but it can frequently appear in those with rheumatoid arthritis (RA). In consultation with a physician, the first part of the diagnosis is to make sure that the patient does not have RA. Occasionally, scleritis may affect the cornea as well. Yellowing does not necessarily mean inflammation, Dr. Schwartzman explains - it could be old inflammation or other problems – but it is important to be seen by a specialist for a proper diagnosis and treatment.
A note about the aforementioned conditions:
Dr. Schwartzman stresses that dry eyes are common in lupus, and the other complications above are less common. If any of the aforementioned symptoms appear, it is important to consult with your rheumatologist and ophthalmologist.
Many eye problems in patients with lupus originate from the drugs doctors use to treat lupus itself.
Three groups of medications prescribed for lupus patients are immunosuppressive drugs (such as methotrexate and mycophenolate), antimalarials (such as hydroxychloroquine (Plaquenil®)), and steroids. “All three of those kinds of drugs can affect the eye adversely,” Dr. Schwartzman explains.
Immunosuppressive agents can increase the risk of infections of the eye, such as conjunctivitis.
Antimalarial medications are some of the safest drugs rheumatologists use. One extremely rare concern in those with lupus who are taking antimalarials, however, is the risk of damage to the retina. Retinal problems that are caused by these drugs are usually seen when large doses are used, and are more common with chloroquine than the much more commonly used hydroxychloroquine (Plaquenil®), but retinal toxicity can occasionally occur. It is important for patients on antimalarial agents to be followed regularly by an ophthalmologist.
Steroids are usually administered to those with lupus intravenously or in pill form. Sometimes, if there’s inflammation in the eye, they’re given topically as drops. “Any one of those three ways of giving the steroids still predisposes you to glaucoma and cataracts,” notes Dr. Schwartzman.
“The thing that worries me the most about lupus in the eye is infections,” Dr. Schwartzman says.
Lupus, he explains, in itself is associated with an increased risk of infections. Secondly, the drugs used to treat lupus suppress your immune system, which adds another risk in terms of infection.
Infections may occur in the eyelid, on the conjunctiva, or in the inside of the eye (including the anterior or posterior chambers). Topical or systemic antibiotics are used for treatment.
“If you have lupus,” Dr. Schwartzman stresses, “any vision problem requires evaluation by a specialist in that it can occur as a consequence of lupus or the therapy for this disease, or just as a consequence of the aging process that can affect people without lupus.”
Learn more about the HSS SLE Workshop, a free support and education group held monthly for people with lupus and their families and friends.
Summary by Mike Elvin