Pathology notes

Apr 16th, 2015
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SJGREN'S SYNDROMEKIVINDU ROBERT AUGUSTINE Its a clinicopathologic entity characterized by:1. Dry eyes (keratoconjunctivitis sicca)2. Dry mouth(xerostomia)SJGREN'S SYNDROME Common in adult females 4th -5th decade of life F:M 9:1 A slowly progressive autoimmune epithelitis It results from immune-mediated destruction of the lacrimal and salivaryglands May occur alone or with other autoimmune diseases Autoimmune diseases associated with this syndrome:EFFECTS Due to the dry eyes there is damage to the eye surface Sticky eyelids ; lack of tears Enlargement of lacrimal glands There is increased risk of tooth decay because of little saliva produced:therefore increased cavities. Difficulty in chewing and swallowing which might require the patient to useexcess fluids Mouth ulcers Parotid enlargementPATHOGENESIS a proposed theory Response to a viral infection or self-RNA containing immunecomplexes,plasmacytoid DC(pDC) are recruited. Inflammation furtherrecruits myeloid DC(mDC),monocytes and T and B cells. IFN- might then lead to maturation autoantigen presenting mDC inpredisposed individuals The local production IFN- might lead to upregulation of IFN-stimulatedgenes, explaining the IFN-signature seen in a subgroup of patients. Autoantigen presenting mDC could stimulate autoreactive T cells ; which inturn could activate autoreactive B cells to produce autoantibodiesP

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