Autoimmune and Cholestatic Liver Disease

• Audio chapter
  Attending-narrated, listen on the commute.
• ABIM-format MCQs
  5-option vignettes with full wrong-answer teaching.
• Study guide
  Tables, decision trees, primary sources.
• AI tutor
  Chapter-grounded, answers the question you're stuck on.

What this chapter covers

• Section 20.1: Sorting AIH, PBC, and PSC

  The three classical immune liver diseases share a common conceptual frame, which is loss of tolerance to a self-antigen, and they separate by the cell type that the immune system attacks.

• Section 20.2: AIH diagnosis, histology, and treatment

  Autoimmune hepatitis is the immune attack on hepatocytes by activated T cells responding to self-antigens that have escaped tolerance, with B-cell-derived autoantibodies serving as serologic markers rather than effectors.

• Section 20.3: PBC diagnosis, histology, and natural history

  Primary biliary cholangitis is a slowly progressive autoimmune cholangiopathy in which T cells attack the small intralobular bile duct epithelium and produce a destructive non-suppurative cholangitis with progressive ductopenia and fibrosis.

• Section 20.4: PBC first-line and second-line therapy

  The PBC treatment algorithm starts with universal use of UDCA, then escalates by biochemical response, with the modern second-line landscape expanded by three FDA-approved agents that target nuclear receptors involved in bile acid homeostasis.

• Section 20.5: PBC pruritus management

  Pruritus is the most common symptom in PBC, frequently precedes biochemical disease, and worsens disability scores more than fatigue in many patients.

• Section 20.6: PSC diagnosis and natural history

  Primary sclerosing cholangitis is the chronic immune-mediated inflammation and fibrotic stricturing of the intra and extrahepatic bile ducts, producing the classic multifocal stricture pattern with intervening normal-caliber duct segments that gives the cholangiogram its beaded appearance.

• Section 20.7: PSC complications and surveillance

  PSC is a premalignant condition with three associated cancers (cholangiocarcinoma, gallbladder cancer, and colorectal cancer in PSC-IBD), each with specific surveillance intervals and modalities.

• Section 20.8: IgG4 sclerosing cholangitis and overlap syndromes

  The fourth pillar of this chapter is the disease that mimics PSC but behaves differently, plus the two overlap syndromes that blur the AIH-PBC-PSC boundaries.

Podcast episodes

1. 01

   Sorting the Immune Liver Diseases and Autoimmune Hepatitis

   The sorting framework and autoimmune hepatitis: separating the three immune liver diseases by antibody pattern and biliary involvement, autoimmune hepatitis as autoantibody-positive interface hepatitis with its type one and type two split, and treatment with corticosteroids plus a steroid-sparing agent.

2. 02

   Primary Biliary Cholangitis

   The mitochondrial-antibody-positive cholestatic disease of the middle-aged woman: the prognostic scores, ursodeoxycholic acid first-line, the second-line landscape after obeticholic acid left the market, and the stepped management of pruritus.

3. 03

   Primary Sclerosing Cholangitis and IgG4 Disease

   The multifocal stricturing disease of the man with inflammatory bowel disease, with no proven medical therapy and surveillance for three cancers, and IgG4-related sclerosing cholangitis that mimics both sclerosing cholangitis and cholangiocarcinoma but responds to corticosteroids.