Primary biliary cholangitis and Primary sclerosing cholangitis

Primary Biliary Cholangitis
(PBC)

Feature

Primary Sclerosing Cholangitis
(PSC)

Chronic autoimmune destruction
of small intrahepatic bile ducts (IHBD)

Definition

Chronic inflammation and fibrosis of
intra (IHBD) & extrahepatic bile
ducts (EHBD)

Mostly women

Gender
Predilection

Mostly men

40–60 years

Age of Onset

20–40 years

– Sjogrens syndrome 
– Rheumatoid arthritis (RA)
– Celiac disease
– Systemic sclerosis

Associated
Conditions

Ulcerative colitis
(in ~70% cases)

 AMA (Anti-mitochondrial
antibody) +ve 

Autoantibodies

 pANCA +ve (nonspecific) 

ALP increases 

Cholestatic
Enzymes

ALP increases 

Normal biliary tree
on MRCP/ERCP

Imaging Findings

Beading appearance of bile ducts
on MRCP/ERCP

Florid duct lesion (intrahepatic)

Liver Biopsy

Onion-skin fibrosis around bile ducts

Low

Risk of
Cholangiocarcinoma

High

Ursodeoxycholic acid (UDCA)

Treatment

Liver transplant (only definitive),
UDCA role limited

Good with treatment

Prognosis

Progressive, risk of cirrhosis
& malignancy