sjogren's syndrome: difficult case presentation: rather than a
TRANSCRIPT
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 FoxScripps [email protected]
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 andANA >640 (SS-A/B+), ESR 20
Positive lip biopsy (focus score 2)
Schirmer's 2 mm, Abnormal Tear Breakup timeMild 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 biopsyone 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 implantedmoisture pump (currently available in Japan)
Stems are water reservoir
Working with designersfor western face
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
M“Moisture shield” glasses and sunglasses
Available at “dryeyezone.com”
Moisture Shields for night or airlineDryeyezone.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
1. Pain often increases with time2. Even though objective measures of disease progression are absent
Summary of issues-1
Summary of issues-2This 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-3SS 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
