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Current issues in the Diagnosis and Management of Sjogren’s Syndrome Robert I. Fox, MD., Ph.D. Scripps Memorial Hospital And Research Institute La Jolla, CA bobfox@ Primary Sjogren A systemic autoimmune disease whose characteristic is ocular and salivary involvement, but also includes other organs such as lung (pneumonitis), kidney (interstitial nephritis), and neurological (central and peripheral) and lymphoproliferative features Goal-1 Correct therapy depends on correct diagnosis New international criteria Potential pitfalls in diagnosis Goals-2 Review the use of Topical medications for dry eyes and dry mouth Goals-3 Review the current guidelines for diagnosis and therapy of extra glandular manifestations Goals-4 How to empower the patient to participate in their own care Epidemiology of Sjogren’s Predominately women (9:1) with two ages of median onset In the 30’s and 50’s 2. Much of what we call SLE in the older patient is actually Sjogren’s syndrome What causes Sjogren’s A combination of Genetic and Environmental Factors From family and twin studies, approximately 4 genes are required but even then an environmental factor is needed Genetics Most important is HLA-DR, which correlates closely with ANA and anti-SS-A antibody Genes of B-cell activation similar to SLE patients Environmental No single agent identified Viral candidates may include EBV and coxsackie viruses Hepatitis C, HIV and HTLV-1 can mimic Objective-1Clinical Issues There is good agreement about diagnosis for the patient with florid symptoms of keratoconjunctivitis sicca (KCS), parotid swelling, and high titer ANA with SS-A/SS-B. The issue in these patients will be therapy And the extent of extra glandular involvement. Typical features of dry eyes, dry mouth and swollen glands Dryness results in the clinical appearance of keratoconjunctivitis sicca (KCS)characteristic of Sjogren’s syndrome Severe Xerostomia with dry tongue Sjogren’s syndromeEye and Oral Features Most of these patients
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