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PRACA PRZEGLĄDOWA
Zespół Sjögrena a choroby IgG4-zależne – trudności w klasyfikacji, postępy w leczeniu
 
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Data nadesłania: 22-03-2014
 
 
Data ostatniej rewizji: 12-07-2014
 
 
Data akceptacji: 25-07-2014
 
 
Data publikacji online: 12-09-2014
 
 
Data publikacji: 31-08-2014
 
 
Reumatologia 2014;52(4):255-262
 
SŁOWA KLUCZOWE
DZIEDZINY
STRESZCZENIE
Zespół Sjögrena (ZS) jest przewlekłą chorobą autoimmunologiczną charakteryzującą się naciekami limfocytarnymi w gruczołach egzokrynnych, głównie ślinowych i łzowych, co doprowadza do upośledzenia ich funkcji. U części chorych występują objawy pozagruczołowe, m.in. przewlekłe zmęczenie, artralgia, zajęcie płuc, nerek, ośrodkowego czy obwodowego układu nerwowego. Ostatnie lata przyniosły zrozumienie niektórych mechanizmów patogenetycznych, dzięki czemu pojawiły się strategie terapeutyczne wpływające na aktywność komórek B. Amerykańskie Towarzystwo Reumatologiczne zaproponowało kryteria klasyfikacyjne ZS oparte na obiektywnych objawach. Choroby IgG4-zależne stanowią nową jednostkę nozologiczną. Trudności diagnostyczne spowodowane są podobieństwami ZS do choroby Mikulicza uznawanej za podtyp choroby IgG4-zależnej. Charakterystycznymi jej cechami jest występowanie zwiększonego stężenia immunoglobulin IgG4 oraz naciekanie narządów miąższowych przez komórki plazmatyczne. Niniejszy artykuł ma na celu przybliżenie klasyfikacji, patogenezy oraz metod terapeutycznych ZS i chorób IgG4-zależnych.
 
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