Women at greater risk for autoimmune
diseases with ocular manifestations
episcleritisBy Katrina Altersitz
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Three times as many women as men experience scleroderma,
or progressive systemic sclerosis, a connective tissue
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
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
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.”