Primary Sjögren syndrome
Synonyms: Primary Sjögren-Gougerot syndrome
A rare systemic autoimmune disease characterized by exocrine gland dysfunction resulting predominately in keratoconjunctivitis sicca and xerostomia but also affecting exocrine glands of the skin as well as respiratory urogenital and digestive tract. Extraglandular manifestations include arthritis interstitial lung disease renal disease and peripheral neuropathy. The disease is accompanied by a substantially increased risk to develop B-cell non-Hodgkin lymphoma especially MALT (mucosa-associated lymphoid tissue) lymphoma.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version December 2023
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Primary Sjögren syndrome?
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