A Case with Sjogren's Syndrome and Behçet's Disease Developing Secondary Amyloidosis

Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic and plasma cell infiltration of lacrimal and salivary glands. Behcet's disease (BD) is a systemic disease characterized by recurrent orogenital apthous ulcerations and eye lesions like iridocyclitis. In this report, we describe a case that has both SS and BD associated with systemic AA type amyloidosis. The patient was a 70-year-old female who has been diagnosed with BD for twenty-two years and SS for ten years. She complained of nausea, vomiting, leg swelling and nocturia for the last few months. Duodenal biopsy was consistent with systemic AA type amyloidosis. Minor salivary gland biopsy demonstrated both systemic amyloidosis and autoimmune sialoadenitis. Both BD and SS can predispose amyloidosis deposition.