Primary Biliary Cholangitis (PBC) (previously called Primary Biliary Cirrhosis but not all develop cirrhosis) is a chronic autoimmune cholestatic liver disease. 💡 Classic triad: middle-aged woman + raised ALP + antimitochondrial antibody (AMA) positive.

📖 About

Autoimmune destruction of intrahepatic small/medium bile ducts → cholestasis → fibrosis → cirrhosis.
90–95% have positive antimitochondrial antibodies (AMA, M2 subtype), specificity ~98%.
Strong female predominance (9:1), usually diagnosed in women aged 40–60.
Associated autoimmune conditions: hypothyroidism, Sjögren’s syndrome, rheumatoid arthritis, systemic sclerosis.

🧬 Pathophysiology / Background

Granulomatous lymphocytic inflammation around bile ducts → progressive ductopenia.
Cholestasis causes accumulation of toxic bile acids → hepatocyte injury.
Chronic inflammation leads to periportal fibrosis and eventually cirrhosis.

🩺 Clinical Features

Often asymptomatic, detected on LFTs (raised ALP, GGT).
Symptoms: fatigue, pruritus, right upper quadrant discomfort.
Later signs: skin hyperpigmentation, xanthelasma/xanthomas, hepatosplenomegaly, jaundice.
Complications: fat-soluble vitamin deficiency (A, D, E, K), osteoporosis, portal hypertension, varices.

🔎 Investigations

LFTs: ALP often >1000 IU/L; GGT raised; AST/ALT mild elevation.
Antibodies: AMA (M2 subtype) positive in 95%; ↑ serum IgM.
Ultrasound: rules out extrahepatic obstruction.
Liver biopsy: shows granulomas + bile duct destruction; not always required if ALP + AMA are diagnostic.
Other: lipid abnormalities (hypercholesterolaemia, xanthomas).

⚕️ Management

First-line: Ursodeoxycholic acid (UDCA) → improves bile acid excretion, slows progression, relieves pruritus.
Second-line: Obeticholic acid (for UDCA-intolerant or non-responders).
Pruritus relief: cholestyramine, rifampicin, naltrexone (stepwise).
Supportive: Fat-soluble vitamin supplementation, manage osteoporosis, nutrition optimisation.
Liver transplantation: excellent outcomes in advanced disease or refractory pruritus.

📉 Poor Prognostic Indicators

Jaundice (raised bilirubin).
Low albumin, prolonged PT/INR.
Presence of ascites, oedema, encephalopathy.
Portal hypertension and variceal bleeding.

🧾 Differentials

Primary Sclerosing Cholangitis (PSC): more common in men, associated with ulcerative colitis, p-ANCA positive, involves extrahepatic ducts (beading on MRCP).
Drug-induced cholestasis.
Biliary obstruction from gallstones or malignancy.

🌟 Summary: PBC is an autoimmune cholestatic liver disease of middle-aged women. Diagnosis = raised ALP + positive AMA. Treatment = UDCA (± obeticholic acid), vitamin support, transplant in advanced cases. Complications = cirrhosis, varices, fat-soluble vitamin deficiency.

📊 Comparison: PBC vs PSC vs Autoimmune Hepatitis

Feature	Primary Biliary Cholangitis (PBC)	Primary Sclerosing Cholangitis (PSC)	Autoimmune Hepatitis (AIH)
Typical Patient	Middle-aged woman	Young/middle-aged man	Young woman (teens–40s)
Association	Other autoimmune (thyroid, Sjögren’s, RA)	Ulcerative colitis (70%), Crohn’s disease	Other autoimmune (thyroid, DM1, coeliac)
Antibodies	AMA (M2) +, ↑ IgM	p-ANCA + (non-specific)	ANA, SMA, anti-LKM1 +, ↑ IgG
LFT Pattern	Cholestatic (↑ ALP, ↑ GGT)	Cholestatic (↑ ALP, ↑ GGT)	Hepatitic (↑ ALT/AST)
Imaging	Normal US (excludes obstruction)	MRCP/ERCP: “beading” of bile ducts	Usually normal biliary imaging
Histology	Granulomas around bile ducts, ductopenia	Onion-skin fibrosis around bile ducts	Interface hepatitis (lymphoplasmacytic)
Complications	Cirrhosis, portal HTN, fat-soluble vitamin deficiency, HCC	Cholangiocarcinoma, cirrhosis, recurrent cholangitis	Cirrhosis, liver failure, HCC (if chronic)
First-line Rx	Ursodeoxycholic acid	Liver transplant (no proven medical therapy)	Immunosuppression (Prednisolone ± Azathioprine)

💡 Exam tip: - PBC → middle-aged woman + ↑ALP + AMA + pruritus. - PSC → young man + UC + ↑ALP + cholangiogram “beading”. - AIH → young woman + ↑ALT/AST + ANA/SMA + responds to steroids.

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Primary Biliary Cholangitis