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.
MeSH terms
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Amyloid / analysis
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Amyloidosis / complications*
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Amyloidosis / diagnosis
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Amyloidosis / immunology
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Bone Marrow Diseases / complications
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Bone Marrow Diseases / diagnosis
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Bone Marrow Diseases / immunology
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Echocardiography
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Electrocardiography
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Female
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Heart Diseases / complications
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Heart Diseases / diagnosis
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Heart Diseases / immunology
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Hematopoietic Stem Cell Transplantation
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Humans
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Immunoglobulin G / analysis
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Middle Aged
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Plasma Cells / pathology
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Salivary Glands / chemistry
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Salivary Glands / pathology
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Sjogren's Syndrome / complications*
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Sjogren's Syndrome / diagnosis
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Sjogren's Syndrome / immunology
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Stomach Diseases / complications
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Stomach Diseases / diagnosis
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Stomach Diseases / immunology