Primary biliary cholangitis (PBC)

(Hep 2019;69:394; Nat Rev 2020;17:93; Lancet 2020;396:1915)

• Autoimmune destruction of intrahepatic bile ducts

• Epi:

  • ♀ 40-60 y;
  • a/w
    • Sjögren (50%),
    • Raynaud phenomenon
    • scleroderma, detial, GOTO: systemic sclerosis and scleroderma disorders
    • celiac & thyroid disease;
  • may be triggered by infxns or toxins;
  • a/w X monosomy, variants in IL12α & IL12R genes

• Sx:

  • fatigue/sleep disturbance,
  • pruritus
  • jaundice
  • 50% asx w/ only LFT abnormalities

• Ddx of Primary biliary cholangitis:

• Dx:

  • ↑ Aϕ,
  • ↑ bili,
  • ↑ IgM,
  • ↑ chol (mainly HDL-C),
  • ⊕ (AMA) in 95%. If ⊕ AMA,
  • liver bx not needed due to high Se & Sp. 0.5% gen pop ⊕ AMA & nl LFTs → 10% develop PBC at ↣ 6 y. If AMA ⊖,
  • liver bx (Pts often ⊕ ANA, smooth muscle Ab; same prognosis as ⊕ AMA).

• Rx-Primary biliary cholangitis (PBC)