Concomitant diagnosis of primary Sjögren's syndrome and systemic AL amyloidosis.
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.
Delèvaux
I
I
Department of Internal Medicine, G Montpied hospital, BP 69, 63003 Clermont-Ferrand, France.
André
M
M
Amoura
Z
Z
Kémény
J L
JL
Piette
J C
JC
Aumaître
O
O
D000682:Amyloid / Q000032:analysis; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D001855:Bone Marrow Diseases / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006331:Heart Diseases / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008875:Middle Aged; D010950:Plasma Cells / Q000473:pathology; D012469:Salivary Glands / Q000737:chemistry / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D013272:Stomach Diseases / Q000150:complications / Q000175:diagnosis / Q000276:immunology
D000682:Amyloid; D007074:Immunoglobulin G