RT Journal Article
SR Electronic
T1 Concomitant diagnosis of primary Sjögren's syndrome and systemic AL amyloidosis
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 694
OP 695
DO 10.1136/ard.60.7.694
VO 60
IS 7
A1 I Delèvaux
A1 M André
A1 Z Amoura
A1 J-L Kémény
A1 J-C Piette
A1 O Aumaître
YR 2001
UL http://ard.bmj.com/content/60/7/694.abstract
AB 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 IgGλ. 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.