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2017 ; 11
(6
): 771-780
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Extrahepatic Manifestations of Primary Biliary Cholangitis
#MMPMID28292174
Chalifoux SL
; Konyn PG
; Choi G
; Saab S
Gut Liver
2017[Nov]; 11
(6
): 771-780
PMID28292174
show ga
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by
progressive destruction of the intrahepatic bile ducts, leading to cholestasis.
PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic
manifestations are seen in up to 73% of patients with PBC, with the most common
being Sjogren's syndrome, thyroid dysfunction and systemic sclerosis. It is
thought that patients with PBC are at increased risk of developing these
extrahepatic manifestations, almost all of which are autoimmune, because patients
with autoimmune disease are at higher risk of developing another autoimmune
condition. Due to the high prevalence of extrahepatic diseases in patients with
PBC, it is important to complete a thorough medical history at the time of
diagnosis. Prompt recognition of extrahepatic disease can lead to improved
patient outcomes and quality of life. The following review summarizes the most
common extrahepatic conditions associated with PBC.