Sjogrens syndrome:Sjogrens syndrome:The Underestimated afflictionThe Underestimated affliction

By Sam HoltonBy Sam Holton

Definition and basic info.Definition and basic info.• SS is characterized by xerophthalmia and xerostomia SS is characterized by xerophthalmia and xerostomia

due to immune-mediated destruction of exocrine due to immune-mediated destruction of exocrine glandsglands

• immune system mistakenly attacks and destroys immune system mistakenly attacks and destroys moisture glands primarily lachrymal and ocularmoisture glands primarily lachrymal and ocular

• Affects around 3,000,000 people in the u.s. alone, the Affects around 3,000,000 people in the u.s. alone, the 22ndnd most common autoimmune disorder most common autoimmune disorder

• 9 out of 10 sufferers are female9 out of 10 sufferers are female• A disproportionate number of SS sufferers have A disproportionate number of SS sufferers have

rheumatoid arthritis and vice versa suggesting basic rheumatoid arthritis and vice versa suggesting basic connectionconnection

• Though not life threatening can be life changing; Venus Though not life threatening can be life changing; Venus Williams couldn’t compete in the 2011 U.S. OpenWilliams couldn’t compete in the 2011 U.S. Open

HistoryHistory

• Potentially first discovered by Mikhail Potentially first discovered by Mikhail Mickulicz in 1882 but description was very Mickulicz in 1882 but description was very generalgeneral

• Henrik Sjogren and his wife Maria discovered Henrik Sjogren and his wife Maria discovered and documented it officially while studying and documented it officially while studying Rheumatoid arthritis in 1930Rheumatoid arthritis in 1930

• Paper wasn’t accepted by the review board in Paper wasn’t accepted by the review board in 1933 (15 subjects) but was later accepted in 1933 (15 subjects) but was later accepted in 1951 when they did larger trials (80 subjects)1951 when they did larger trials (80 subjects)

• 1982 Elaine K. Harris founded the Sjogrens 1982 Elaine K. Harris founded the Sjogrens Syndrome foundationSyndrome foundation

SymptomsSymptoms

Biomolecular basis Biomolecular basis • Several overlapping Several overlapping theoriestheories

• Most center around Most center around cytokine environmentcytokine environment

• TNF TNF αα and Interleukins 1 and Interleukins 1 and 21and 21

• IFN IFN αα• Mutated HLA’sMutated HLA’s• Aquaporin-7 dysfunctionAquaporin-7 dysfunction• Study of saliochemistry Study of saliochemistry

showed women with SS showed women with SS had more Cl, K and Ca had more Cl, K and Ca ions in salivaions in saliva

• Intracellular adhesion Intracellular adhesion dysfunction in salivary dysfunction in salivary glandsglands

Genetic/environmental basisGenetic/environmental basis• D3 increases risk fourfoldD3 increases risk fourfold• Muc-16 related to xeropthalmiaMuc-16 related to xeropthalmia• La/SS-b protein created due to La/SS-b protein created due to

alternative splicing of exon-1 out of the alternative splicing of exon-1 out of the La geneLa gene

• R.A. is closely relatedR.A. is closely related• Close relative with SS gives one 20:1 Close relative with SS gives one 20:1

odds of having SSodds of having SS• Having Certain histocompatability Having Certain histocompatability

antigensantigens• Epstein-Barr virus, Hepatitis-C and Epstein-Barr virus, Hepatitis-C and

Human T-cell leukemia virus-1 all raise Human T-cell leukemia virus-1 all raise risk of getting SSrisk of getting SS

• Hormonal differences between men Hormonal differences between men and womenand women

Diagnosis and treatmentDiagnosis and treatment• Sjogrens has no known cure Sjogrens has no known cure but the symptoms can be but the symptoms can be alleviated with religious alleviated with religious application of topical application of topical moisturizers, NSAIDs ect.moisturizers, NSAIDs ect.

• Diagnosed usually with eye Diagnosed usually with eye dryness (Shirmer) tests, Rose-dryness (Shirmer) tests, Rose-Bengal dye, lip assays or blood Bengal dye, lip assays or blood workwork

• Usually requires multiple Usually requires multiple different tests for diagnosisdifferent tests for diagnosis

• Diagnosis is mainly a last Diagnosis is mainly a last resort and is often confused resort and is often confused with chronic fatigue syndrome, with chronic fatigue syndrome, depression, menopause or depression, menopause or multiple sclerosismultiple sclerosis

New researchNew research• 4/18/12: researchers at Peking University 4/18/12: researchers at Peking University

Peoples Hospital analyzed incidence rates Peoples Hospital analyzed incidence rates of certain symptomsof certain symptoms

• 5/6/12: Department of Rheumatology and 5/6/12: Department of Rheumatology and Clinical Immunology, UMC Utrecht tested Clinical Immunology, UMC Utrecht tested efficacy of Leflunomide; able to decrease efficacy of Leflunomide; able to decrease levels of IL-4 and TNFlevels of IL-4 and TNFαα ( inhibition of T- ( inhibition of T-helper cell 1 cytokine release)helper cell 1 cytokine release)

• 3/23/12: Experimental Laboratory 3/23/12: Experimental Laboratory Immunology, Catholic University Leuven Immunology, Catholic University Leuven used Antinuclear antibody immunoassays used Antinuclear antibody immunoassays to detect SS along with similar diseasesto detect SS along with similar diseases