Keratitis is another ocular manifestation of lupus. Patients can develop peripheral keratitis or multifocal superficial corneal inflammation, leaving small nebulae in the superficial cornea stroma.
“Both of them are associated with light sensitivity and photophobia. Both are important because they both carry with them a little more prognostic significance than does episcleritis or conjunctivitis, “Both are fairly disabling because of the photophobia.” Corticosteroids are usually used for treatment, but the long-term solution for keratitis is to address the underlying lupus with systemic medication.
“The Episcleritis is first identification of the systemic disease associated with it.
Plaquenil (hydroxychloroquine, Sanofi Winthrop), the traditional systemic treatment for lupus, can cause damage to the retina when used in high dosage.
“There is a danger dose, which is measured in milligrams per kilogram, so a smaller person would be more likely to be at that danger dose using a regular dose of Plaquenil, “It causes a retinopathy, basically dysfunction of the retina cells.”
The site for some of the most serious ocular complications of lupus is the retina.
“Patients with lupus can develop an immune complex vasculitis with the lodging of immune complexes forming in the vasculature, both in the choroid and in the retinal vasculature itself,” he explained.
This ophthalmic emergency can cause permanent vision loss, but it can also be a prompt for systemic lupus testing in an as yet undiagnosed patient or a sign of similar immune complexes in other areas of the body.
Rheumatoid arthritis, another collagen vascular disease, is four times more common in women than men and presents problems similar to those with lupus, including dry eye.
“You can also get scleritis and pretty severe inflammatory disorders, things like melting of the peripheral cornea,” Dr. Colby said. “There’s a lot of overlap with lupus.”
Dr. Foster said the approach to treating dry eye or secondary Sjögren’s in rheumatoid arthritis patients is much like that in lupus, including the use of Restasis (cyclosporine ophthalmic emulsion, Allergan) along with heat and massage to improve the function of the meibomian glands.
In addition, he said, the ophthalmologist must be aware of the patient’s systemic disease management to best treat its ocular manifestations.
“See if there is any evidence of the underlying rheumatoid disease being active, so to speak, even if it’s not active in the joints,” Dr. Foster said. “Are there markers in the blood that would indicate that chronic low-grade inflammatory activity is afoot? If so, deal with that systemically with a sprucing up of the systemic medication program.”
Dr. Foster said that scleritis and peripheral keratitis are all “absolutely dreadful” ocular manifestations of rheumatoid disease that require systemic therapy, often aggressive.
Dr. Colby added, “The important thing with the systemic diseases is, the better the systemic diseases are managed, the easier it is to manage the ophthalmic manifestations. I can treat someone’s rheumatoid-associated dry eye all I want, but if their systemic rheumatoid disease is not under control it’s like sticking your finger in the dike.”
Three times as many women as men experience scleroderma, or progressive systemic sclerosis, a connective tissue disease.
C. Stephen Foster
“Scleroderma is a very different beast from lupus and rheumatoid disease, with less known about it and less discovered thus far in terms of a routinely highly effective therapy,” Dr. Foster said. “Breakthroughs are being made, and there are increasing levels of optimism among the medical community with respect to being able to prevail over some of the more relentless manifestations of scleroderma.”
Patients with scleroderma are likely to develop dry eye, which is treated as in other autoimmune diseases. The condition also causes shrinkage of areas of the skin, including the conjunctiva, Dr. Foster said.
Choroidopathy and vascular occlusion can also occur.
“Just as in lupus, this is a wake-up call that the patient’s scleroderma has taken a nasty turn,” Dr. Foster said.
Hyperthyroiditis affects women seven times as often as men. Its main ocular manifestation is Graves’ orbitopathy.
“That often is relatively mild and basically cosmetic,” Dr. Foster said, “but it is graded and assigned different grades, with severity and vision threat being greater the higher the grade of the thyroid eye disease is.”
Dr. Colby explained that in Graves’ orbitopathy infiltration of inflammatory cells within the extraocular muscles causes proptosis.
“You can have exposure of the cornea, which can lead to corneal ulcers. More seriously, if the muscles are very infiltrated, “crowding” of the orbit can compress the optic nerve of the eye, which can cause vision loss,” she said. “Graves’ disease can also cause double vision because if the muscles are very infiltrated they don’t move properly.”
Mild exposure can be treated with ointment or taping of the lids,
Working with other physicians
“With any of those diseases, the systemic management is really key,” Dr. Colby said. “Often if the eye is out of control, it means the body is out of control, even if the patient is not having systemic symptoms.”
She suggested that ophthalmologists make sure to speak clearly with collaborating physicians, using terms that the other specialist will understand. “Communication is critical,” .