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Diagnosis and Management of Sjogren s Syndrome LCDR Paul Kruszka, USPHS

What is Sjogren's Syndrome. Systemic autoimmune disease characterized by dry eyes and dry mouth.Other organ systems often affected (extra-glandular manifestations)May be primary - solitary processSecondary disease accompanies another autoimmune disease - most often rheumatoid arthritis or SLE. Gl

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Diagnosis and Management of Sjogren s Syndrome LCDR Paul Kruszka, USPHS

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    1. Diagnosis and Management of Sjogren’s Syndrome LCDR Paul Kruszka, USPHS/USCG Paul.S.Kruszka@uscg.mil

    2. What is Sjogren’s Syndrome Systemic autoimmune disease characterized by dry eyes and dry mouth. Other organ systems often affected (extra-glandular manifestations) May be primary - solitary process Secondary disease accompanies another autoimmune disease - most often rheumatoid arthritis or SLE

    3. Glossary Xerostomia Xerophthalmia Keratoconjunctivitis sicca : KCS Sialadenitis

    4. Epidemiology Sjogren Syndrome is the third most common autoimmune disease The reported prevalence is between 0.05 and 4.8%1 A study from Olmsted, MN found physician diagnosed cases to be approximately 4 per 100,000 persons.1 1. Pillemer SR, Matteson EL, Jacobsson LT, et al. Incidence of physician-diagnosed primary Sjogren syndrome in residents of Olmsed County, Minnesota. Mayo Clin Proc. 2001;76(6):593-599.

    5. Who gets Sjogren Syndrome A cohort of 400 patients found that the average age of the patients was 52.7 and 93% of the patients were women.2 An estimated 1 to 2 million Americans have Sjogren Syndrome.1 Primary vs. secondary 2. Garcia-Carrasco M, Ramos-Casals M, Rosas J, et al. Primary Sjogren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine. 2002;81(4):270-280.

    6. Pathogenesis Obscure Primary Sjogren syndrome is associated with HLA-DR3; RR = 103 The histologic hallmark: lymphocytic infiltration of exocrine glands leading to gland degeneration, necrosis, and atrophy4 Evidence of B cell role: auto-antibodies to self antigens Mitchell RS, Kumar V, Abbas AK, Fausto N. Robbins Basic Pathology 8th edition. Philadelphia 2007. 4. Cummins MJ, Papas A, Kammer GM, Fox PC. Treatment of Primary Sjogren’s syndrome with low-dose human interferon alfa administered by the oromucosal route: combined phase III results. Arthritis Rheum. 2003;49(4):585-593.

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