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Women at greater risk for autoimmune diseases with ocular manifestations

 episcleritisBy Katrina Altersitz     Page-3

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Three times as many women as men experience scleroderma, or progressive systemic sclerosis, a connective tissue disease.

C. Stephen Foster, MD
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.

“Patients sometimes develop shrinkage of the conjunctiva with a shortening of the inferior fornix,” he said. “It probably has to do with laying down new collagen, perhaps type 3 collagen or embryonic collagen. This contracts and you get shrinkage.”

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.

Unfortunately, he said, there is no treatment for conjunctival shrinkage, and clinical trials for treatment of vascular occlusions are “maddeningly difficult” because of the small number of patients in any one center.

“One could speculate that the same approach that has been employed in the scleroderma lung research, namely with high dose cyclophosphamide therapy intravenously and other kinds of aggressive immunosuppressive therapy, might be beneficial in patients who are developing the scleroderma choroidopathy, but frankly it’s not known,” 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, Dr. Colby said. More advanced disease may require partial tarsorrhaphy. In extreme cases, orbital decompression surgery may be called for.

Dr. Foster said, “As the space occupied by the infiltrating cells, the proliferating fibroblasts, steadily increases, there is only so much space in the orbit, and the optic nerves can get increasingly compressed. That’s something that is typically tackled surgically with decompression surgery.”

Working with other physicians

Given that ocular manifestations of autoimmune disease are related to the status of the systemic disease, Drs. Foster and Colby said ophthalmologists must maintain relationships with the patient’s ocular immunologist or rheumatologist during treatment.

“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.”

“The patient, from a rheumatologist’s standpoint, may still be in remission,” Dr. Foster said. “The ophthalmologist is duty-bound, in my judgment, to convince that rheumatologist that this is not so – that the eye, although it may seem trivial, is extremely important and is telling you, ‘Wake up, pay attention, increase the aggressive therapy.’ If you don’t, you’re going to be sorry 6 months from now because the kidney or the lung is going to be in trouble.”

Thanks Folks