Sjogren's Syndrome:
Difficult Case Presentation:
Rather than a zebra, I will present a common scenario
that often falls in the lap of the rheumatologist
(the only one who will listen to the patient)
Robert Fox
Scripps Memorial-Ximed
robertfoxmd@mac.com
Ms. PT is a 47 yr female software CFO with a
15 yr history of Sjogren's
She is having increasing ocular pain that now is limiting
her ability to use computer and to travel on airplanes
Brief history
15 yr ago-dx SS with dry eyes/mouth and
ANA >640 (SS-A/B+), ESR 20
Positive lip biopsy (focus score 2)
Schirmer's 2 mm, Abnormal Tear Breakup time
Mild KCS and no erosions on slit lamp
Although her ocular and oral pain is now much more severe,
her labs and ocular exam are essentially unchanged from 10 years
ago. She even had a repeat lip biopsy
one year ago (as part of study) and still has focus score 2
Current Treatment
•
Preservative free tears (refresh) and ocular lubricant
(Refresh PM)
•
Prior punctal plugs
•
Restasis (topical cyclosporin)
•
Ophthalmologist has thrown up his hands
Key points
•
On exam, she has blepharitis-in part from overuse of her nighttime
lubricant
Current Plan:
•
Use no more than 1/8 inch lubricant to avoid plugging meibonian
glands and use lid scrubs in am to remove
•
A short course of oral doxycycline was helpful
•
Systane is a useful tear in SS patients with blepharitis
Environmental Factors-1
•
Her work involves 10 hrs./day at computer
•
Blink rate goes down 90% using computer
Current Plan:
Computer glasses and humidification of work area
Environmental Factors-2
•
She travels by airplane frequently (low humidity) and
arrives with eyes in severe pain
•
Use of lubricant or Tranquil-eye (Amazon) helps
•
May require Lotemax (a soft steroid) for a day or two
•
Already in Japan, eyeglass frames with small humidity
pumps
Jinn Glasses with implanted
moisture pump (currently available in Japan)
Working with designers
for western face
Stems are water reservoir
Environment-3
•
She travels to Asia where dry, polluted, and people
smoke
•
Use of sunglasses with moisture shields can be
fashionable and allow outdoor exercise (Wiley)
(dryeyezone.com)
•
Moisture shields on glasses
“Moisture shield” glasses and sunglasses
M
Available at “dryeyezone.com”
Moisture Shields for night or airline
Dryeyezone.com
Things they may not tell you
•
She had a blepharoplasty (facelift around eyes) two
years ago when symptoms exacerbated
•
This frequently leads to exposure keratitis (esp at
night when poor lid apposition)
•
Use of taping lid at night and humidifier
•
Be alert to thyroid exophthalmia
•
Lasik surgery is contra-indicated
Medications
•
She was taking Benadryl (otc) to sleep across time
zones
•
Also some Elavil (from her friend) helped her sleep
•
Especially at night, anticholinergic exacerbate the
diurnal rhythm of decreased secretion at night
•
Many herbal mixtures (otc) are anticholinergic
Although little progression of her biopsy or
exam, her symptoms were dramatically
increased
•
Pain could only decrease from 9 to 6 (on scale of
10) after topical anesthetic
•
This indicates role of nociceptive pain-literally a
corneally mapped pain in prefrontal cortex as a
result of chronic afferent stimuli
•
May respond to combo low dose duloxetene
(Cymbalta) plus pregabalin (Lyrica)
•
Occasionally to low dose naltrexone
Summary of issues-1
1. Pain often increases with time
2. Even though objective measures of
disease progression are absent
Summary of issues-2
This increase in pain is only partly reversed
by Opthaine (topical anesthetic), so e know
it is noci-ceptive (cortically mapped)
New approaches by Neurologists and Pain
Specialists are concentrating on
mechanisms of “veto-neurons” to reverse
this cortically mapped pain
Summary of issues-3
SS provides an interface of immunology
and neurology to study the factors that
relate to pain and fatigue
This is the new frontier for the next decade