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  • Title: Concomitant diagnosis of primary Sjögren's syndrome and systemic AL amyloidosis.
    Author: Delèvaux I, André M, Amoura Z, Kémény JL, Piette JC, Aumaître O.
    Journal: Ann Rheum Dis; 2001 Jul; 60(7):694-5. PubMed ID: 11406525.
    Abstract:
    A 48 year old woman was referred to hospital for buccal discomfort. Physical examination showed a macroglossia and features of xerostomia. She was diagnosed as having primary Sjögren's syndrome according to the criteria proposed by the European Community study group in 1993. Furthermore, a lower lip salivary gland biopsy showed amyloid deposits that were also seen in the stomach and in the bone marrow. Echocardiography was consistent with cardiac amyloidosis. Serum immunofixation identified a monoclonal IgGlambda. As far as is known, this is the first report of systemic primary amyloidosis associated with Sjögren's syndrome. The relation between these two disorders is discussed.
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