Pierwotny zespół Sjögrena

Kod Orpha: 289390Kod OMIM: 270150

Definicja

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.

Dane
Klasyfikacja

Choroba

Synonimy
Primary Sjögren-Gougerot syndrome
Pierwotny zespół Sjögrena-Gougerota
Kod ORPHA
289390
Kod OMIM
270150
Kod ICD10
M35.0
Kod ICD11
4A43.20

Brak opisu rozszerzonego dla tej choroby. Opracowanie w toku.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl