Concise report
Concomitant diagnosis of primary Sjögren's syndrome and
systemic AL amyloidosis
I Delèvauxa, M Andréa, Z Amourac, J-L Kéményb, J-C Piettec, O Aumaîtrea
a Department of
Internal Medicine, G Montpied hospital, BP 69, 63003 Clermont-Ferrand,
France, b Department of Pathology, G Montpied hospital, c Department of Internal
Medicine , Pitié-Salpêtrière hospital, 47-83 boulevard de
l'Hôpital, 75651 Paris cedex 13, France
Correspondence to: Professor O Aumaître oaumaitre{at}chu-clermontferrand.fr
Accepted for publication 15 November
2000
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 IgG
. 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.
© 2001 by Annals of the Rheumatic Diseases
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