Download The management of a 64 year old hispanic women with the use of a

TITLE: Treatment of Severe Aqueous Deficient Dry Eye Secondary to Sjögren’s
Syndrome with Scleral Contact Lenses
AUTHORS: Anita Ticak, OD, MS, Norman Leach OD, MS, FAAO
Management of a 64 year old Hispanic woman with the use of scleral contact lenses in
the treatment and visual enhancement of her severely aqueous deficient dry eye
secondary to Sjögren’s syndrome.
CASE HISTORY
The patient is a 64 year old Hispanic woman with no previously significant ocular
conditions. The patient reported to the University Eye Institute (UEI) with a chief
complaint of worsening red eyes over the past eight months with associated burning,
itching, photophobia and a yellow discharge. She reported visiting an optometrist one
month prior and was told to use Optivar twice a day, artificial tears and lid scrubs for
ocular allergies. She felt a mild reduction in symptoms with use of Optivar. She denied
any significant ocular or medical history, although it was noted she was taking fosamax
but she denied knowledge of osteoarthritis. Medications at the time were Optivar twice a
day, artificial tears as needed, Cingulair, and Fosamax. There was no significant family
history.
PERTINENT FINDINGS AND DIFFERENTIALS
Upon examination the patient’s best corrected visual acuity at distance was 20/30
in the right eye and 20/25 in the left. She was found to have significant superficial
punctate keratitis worse in the right eye (grade 3) than the left (grade 2+), and painful
mucous filament strands on the cornea. There was no visible tear meniscus and a trace
follicular reaction in both eyes. A Schirmer II test (with anesthetic) measured 0 mm OD
and 2 mm OS. Internal ocular health was unremarkable. Upon further questioning the
patient confirmed dry nasal passages and difficulty in production of saliva. She also later
stated difficulty in producing tears when emotionally upset. The diagnosis at this visit
was severe aqueous deficient dry eye, possibly secondary to Sjögren’s syndrome, and
filamentary keratitis.
The filaments were manually removed and the patient was placed on a regimen of
Zylet 1 drop every four hours for inflammation, refresh non-preserved artificial tears
every hour, and non-preserved celluvisc when needed. A blood work up for rheumatoid
arthritis and Sjögren’s syndrome was ordered.
Two and a half months passed in the time to retrieve blood work and the patient’s
vision and comfort continued to decline. During the time span she regularly visited the
clinic every 2-4 weeks and her condition continued to deteriorate. Filaments were
removed as needed and the patient was urged to continue her drop regimen. During this
time she also saw a corneal specialist who further diagnosed ocular rosacea, and
blepharitis. The patient had smart plugs installed, was put on Restasis, and also began
bacitracin as needed for the rosacea. At the two and a half month visit when the results
returned the corneal specialist also placed the patient on a compounded steroid known as
sulomedrol to be used every two hours in both eyes for the first week then tapered to four
times a day for the following 3 weeks. At this visit the patient also reported taking
Celebrex for general body pain and Zoloft for depression. Blood tests revealed a positive
RA factor, + ANA and an increased sedimentation rate confirming the diagnosis of
rheumatoid arthritis with a secondary Sjögren's syndrome. The patient’s vision had fallen
to a best corrected visual acuity of 20/200 right eye and 20/40 left eye only two months
after initial diagnosis. The patients ocular regimen at this time involved refresh artificial
tears (non-preserved) as needed, Restasis twice a day, sulomedrol every two hours for
one week and then four times a day for the next three weeks, celluvisc at night, and
regular visits to manage filament strands and worsening flare ups.
Two weeks later the patient noted a huge improvement and best corrected visual
acuity had improved to 20/50 right eye and 20/30 left eye. The patient still complained of
ocular discomfort and painful photophobia. She continued as before and was told to
return for a one month follow-up. One month later she returned and had taken to
constantly wearing sunglasses indoors and out and continued to remark on the stabbing
pain in her right eye that occurred on and off. Her best corrected visual acuity at this time
was back down to 20/200 right eye and 20/80 in the left eye. The eyes still exhibited
similar findings and it was thought at this time the patient may benefit from some sort of
contact lens bandage for management of the condition. The patient was told to continue
with current medications and to return to clinic in two weeks for follow up. Ocular
medications remained the same (with sulomedrol on a four times a day dosage) and the
addition of voltaren twice a day. The patient no longer took celebrex, and had begun
taking advair and hydrochloroquine.
DIAGNOSIS AND DISCUSSION
Sjögren’s syndrome is an autoimmune disease that is often characterized by
dysfunction of lacrimal and salivary glands resulting in keractoconjunctivitis sicca and
xerostomia. The condition often manifests in later life, typically the fifth decade. The
disease typically presents with a dry eye, which may subjectively present as burning,
itching, and failure to produce tears. Objectively the observer will see a decreased
Schirmer test, decreased tear break up time, minimal tear meniscus and staining of the
cornea and conjunctiva. The disease may even lead to severe filamentary keratitis and
corneal erosion. Typical initial treatment is that of local therapies for the dry eye
syndrome. As the disease progresses often hydroxyquinolone is used, and for more
advanced cases a steroid or other immunsupressive agents may be used. Patients are
prone to flare ups of the condition that require more medical attention. In the case of this
patient, she had very severe and dramatic recurrences of filamentary keratitis, and was at
such a photophobic extreme that she constantly worse sunglasses. This is a common
rheumatic autoimmune disease and may affect upwards of 2-4 million people in the US.
It may be a primary disorder, or as in the case of our patient a secondary disorder to
rheumatoid arthritis. It has also been found to be secondary to lupus erythematosus,
scleroderma, or billiary cirrhosis.
TREATMENT/MANAGEMENT
Due to a continuing decrease in vision and comfort, a contact lens modality was
attempted. The patient was first put into a soft silicone hydrogel lens to serve as a
bandage lens. At first she noticed a mild decrease in discomfort and pain, but after a few
days the lens would become too dry and fall out of the eye. The patient was then fit with
a fenestrated mini scleral design lens and told to fill the lens bowl with refresh nonpreserved tears prior to insertion. The patient was able to achieve 20/25 with each eye
and noted improvement in ocular symptoms but had difficulty wearing the lenses more
than a few hours at a time before discomfort began to occur. The next lens design
attempted was a non-fenestrated mini-scleral design to help increase the length of time
the cornea was exposed to the Refresh tears. On initial fitting and dispense the patient
found the lenses to be very comfortable and vision again was 20/25 with each eye. The
scleral contact lens is designed to vault the cornea and rest upon the sclera thereby
providing improved comfort. This also allows for the bowl to be filled with solution
(such as the non-preserved refresh tears) so that the cornea is bathed in fluid and acts as a
liquid bandage. Studies have shown that wearing such lenses decrease pain and
photophobia and improve overall quality of life. The patient is also being referred to a
center that does work with autologus tears. Autologus tears may be beneficial because
they contain a number of growth factors which are needed for the proliferation,
migration, and differentiation of corneal epithelial cells. This may be what is used as her
future liquid agent when wearing the scleral lenses. The patient is due for follow up in the
near future.
CONCLUSION
Patients with Sjögren’s syndrome can rapidly deteriorate to a state of poor vision, severe
discomfort, and debilitating photophobia. This patient is a caretaker and enjoys the
simple acts of reading and sewing and felt her quality of life had fallen dramatically. It is
important for optometrists as primary eye care professionals to stay up to date on the
latest options available and undertake the management of these patients. It is also
important to address that patient’s issues and get creative. When dealing with a severe
dry eye a scleral lens design may in fact be a patient’s best option, and helping the patient
find a system that works for them (such as lens design and artificial tears in the bowl)
may make all the difference.
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