Sjögren’s Syndrome

Posted by e-Medical PPT Thursday, April 12, 2012
Sjögren’s (show-grin) syndrome - a syndrome describing xerophthalmia (dry eyes) and xerostomia (dry mouth)- (Sicca complex)-due to immune-mediated destruction of exocrine glands, predominately of lacrimal and salivary. Primary Sjögren’s syndrome - characterized by Sicca complex and extra-glandular symptoms without any additional connective tissue disorder. Secondary Sjögren’s syndrome occurs in association with another autoimmune disorder such as SLE, RA, or scleroderma Primary Sjögren’s Syndrome has a ratio 9:1 of women to men Age range from 40-60, with mean of 52.7 years. However, case reports have been seen in children. Pathogenesis of Sjögren’s syndrome is believed to be multifactorial. Known to be autoimmune, but studies suggest that the disease process has genetic, environmental and neuroendocrine components There is a genetic predominance of HLA-DR genotype in SS patients. HLA-DR is a major histocompatibility complex, MHC class II cell surface receptor. It is found on antigen presenting cells. These genotypes may also produce dysfunctional glands that secrete abnormal amounts of immune-stimulatory chemokines. This is not seen in patients without Sjögren’s disease. Autoimmunity involved in Sjögren’s disease is still poorly understood. It is believed that genetically defective glandular tissue combined with immunologic, environmental or neuroendocrine factors leads to loss of self antigen awareness. Environmental factors may have link to viruses (Epstein–Barr virus, hepatitis C virus, human T-cell leukemia virus-1) After the initial trigger, this glandular tissue autoimmune complex then becomes infiltrated with lymphocytes, predominately CD4 T cell (attracted by MHC class II) A cascade of events then occur with the CD4 T cell release of cytokine IL-1, TNF, and interferon-gamma, which have destructive effects on the tissue and interfere with acetylcholine release, causing dysfunctional glands. Clinical Manifestations Glandular manifestations Decreased lacrimal flow Corneal and conjunctiva epithelia damage Leads to dry eyes, foreign body sensation, irritation, photosensitivity, thick secretions at inner canthus, and visual impairment. Involvement of unilateral or bilateral major and minor salivary glands Decreased salivary secretions-loss of lubrication, buffering and antimicrobial capacities of saliva. Most common complication is increase in dental caries, especial root and incisor caries. Frequent fungal infections Tongue fissures Persistent salivary enlargement...

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