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Sjogrens syndrome

:
The Underestimated affliction
By Sam Holton

Definition and basic info.
• SS is characterized by xerophthalmia and xerostomia
due to immune-mediated destruction of exocrine glands
• immune system mistakenly attacks and destroys
moisture glands primarily lachrymal and ocular
• Affects around 3,000,000 people in the u.s. alone, the
2
nd
most common autoimmune disorder
• 9 out of 10 sufferers are female
• A disproportionate number of SS sufferers have
rheumatoid arthritis and vice versa suggesting basic
connection
• Though not life threatening can be life changing; Venus
Williams couldn’t compete in the 2011 U.S. Open

History
• Potentially first discovered by Mikhail Mickulicz in
1882 but description was very general
• Henrik Sjogren and his wife Maria discovered
and documented it officially while studying
Rheumatoid arthritis in 1930
• Paper wasn’t accepted by the review board in
1933 (15 subjects) but was later accepted in
1951 when they did larger trials (80 subjects)
• 1982 Elaine K. Harris founded the Sjogrens
Syndrome foundation

Symptoms
Biomolecular basis
• Several overlapping
theories
• Most center around
cytokine environment
• TNF α and Interleukins 1
and 21
• IFN α
• Mutated HLA’s
• Aquaporin-7 dysfunction
• Study of saliochemistry
showed women with SS had
more Cl, K and Ca ions in
saliva
• Intracellular adhesion
dysfunction in salivary glands
Genetic/environmental basis
• D3 increases risk fourfold
• Muc-16 related to xeropthalmia
• La/SS-b protein created due to
alternative splicing of exon-1 out
of the La gene
• R.A. is closely related
• Close relative with SS gives one
20:1 odds of having SS
• Having Certain histocompatability
antigens
• Epstein-Barr virus, Hepatitis-C
and Human T-cell leukemia
virus-1 all raise risk of getting SS
• Hormonal differences between
men and women
Diagnosis and treatment
• Sjogrens has no known cure
but the symptoms can be
alleviated with religious
application of topical
moisturizers, NSAIDs ect.
• Diagnosed usually with eye
dryness (Shirmer) tests, Rose-
Bengal dye, lip assays or blood
work
• Usually requires multiple
different tests for diagnosis
• Diagnosis is mainly a last
resort and is often confused
with chronic fatigue syndrome,
depression, menopause or
multiple sclerosis
New research
• 4/18/12: researchers at Peking University
Peoples Hospital analyzed incidence rates of
certain symptoms
• 5/6/12: Department of Rheumatology and
Clinical Immunology, UMC Utrecht tested
efficacy of Leflunomide; able to decrease levels
of IL-4 and TNFα ( inhibition of T-helper cell 1
cytokine release)
• 3/23/12: Experimental Laboratory Immunology,
Catholic University Leuven used Antinuclear
antibody immunoassays to detect SS along with
similar diseases