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 Memorial-Ximedrobertfoxmd@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 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-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