Concomitant diagnosis of primary Sjogren's syndrome and systemic AL amyloidosis

A 48 year old woman was referred to hospital for buccal discomfort. Physica l examination showed a macroglossia and features of xerostomia. She was dia gnosed as having primary Sjogren's syndrome according to the criteria propo sed by the European Community study group in 1993. Furthermore, a lower lip salivary gland biopsy showed amyloid deposits that were also seen in the s tomach and in the bone marrow. Echocardiography was consistent with cardiac amyloidosis. Serum immunofixation identified a monoclonal IgG lambda. As f ar as is known, this is the first report of systemic primary amyloidosis as sociated with Sjogren's syndrome. The relation between these two disorders is discussed.