Primary Biliary Cholangitis vs Primary Sclerosing Cholangitis
Primary Biliary Cholangitis and Primary Sclerosing Cholangitis are easy to mix up on the boards. Here's a side-by-side comparison — presentation, workup, imaging, and first-line treatment — drawn from our full outlines.
Primary Biliary Cholangitis vs Primary Sclerosing Cholangitis at a glance
- Primary Biliary Cholangitis: Autoimmune destruction of small intrahepatic bile ducts → cholestasis, pruritus, fatigue; AMA-positive in 95%.
- Primary Sclerosing Cholangitis: Chronic fibroinflammatory disease of intra- and extrahepatic bile ducts → strictures and biliary cirrhosis; strong IBD link.
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Side-by-side comparison
| Feature | Primary Biliary Cholangitis | Primary Sclerosing Cholangitis |
|---|---|---|
| At a glance | Autoimmune destruction of small intrahepatic bile ducts → cholestasis, pruritus, fatigue; AMA-positive in 95%. | Chronic fibroinflammatory disease of intra- and extrahepatic bile ducts → strictures and biliary cirrhosis; strong IBD link. |
| Classic presentation | Middle-aged woman with fatigue, pruritus, and isolated alkaline phosphatase elevation — check AMA.; Often asymptomatic at diagnosis (incidental cholestatic LFT pattern in ~60%); Fatigue (the most common symptom, often disabling); Pruritus (often nocturnal, worsens before jaundice); Right upper quadrant discomfort; Sicca symptoms (dry… | Young man with ulcerative colitis and an elevated alkaline phosphatase — get an MRCP.; Often asymptomatic at diagnosis (~50%) — found via incidental cholestatic LFTs in known IBD; Fatigue, pruritus, right upper quadrant pain; Intermittent jaundice and recurrent bacterial cholangitis (fever, RUQ pain, jaundice — Charcot triad); Weight… |
| Workup / key labs | 2 of 3: (1) cholestatic biochemistry (elevated alkaline phosphatase ≥1.5× ULN >6 months), (2) AMA positive (≥1:40) or PBC-specific ANA, (3) liver biopsy showing nonsuppurative destructive cholangitis with bile duct injury. Biopsy not required if first two are met.; LFTs — cholestatic pattern: elevated alkaline phosphatase and GGT,… | Characteristic MRCP/ERCP findings + cholestatic LFTs + exclusion of secondary causes. Small-duct PSC requires biopsy with cholestatic biochemistry and IBD.; Cholestatic LFTs: elevated alkaline phosphatase, GGT, modest AST/ALT; bilirubin variable, elevates with strictures or cirrhosis; p-ANCA positive in ~70% (atypical perinuclear… |
| Imaging | Abdominal ultrasound — exclude biliary obstruction; nonspecific for PBC; MRCP — typically normal in PBC (distinguishes from PSC, which shows beading) | Magnetic resonance cholangiopancreatography (MRCP) — first-line diagnostic test; demonstrates multifocal intra- and extrahepatic strictures with alternating dilation ('beaded' appearance); ERCP — therapeutic role for dominant strictures; reserved for intervention or when MRCP nondiagnostic; Liver biopsy — not required for diagnosis if… |
| First-line treatment | Ursodeoxycholic acid (UDCA) 13-15 mg/kg/day in divided doses — first-line for all PBC patients; improves transplant-free survival; Assess biochemical response at 12 months: Paris II / Toronto criteria — alkaline phosphatase reduction to <1.67× ULN and normal bilirubin | No proven disease-modifying medical therapy; Ursodeoxycholic acid (UDCA) — improves biochemistry but no proven survival benefit; high-dose (28-30 mg/kg/day) is harmful (increased adverse events, do NOT use); moderate dose (13-15 mg/kg/day) is commonly used but controversial — AASLD does not routinely recommend; Symptomatic pruritus:… |
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