Show notes
Episode one of the Autoimmune and Cholestatic Liver Diseases chapter builds the sorting framework for the three immune liver diseases and then works autoimmune hepatitis in depth. The organizing idea: pattern plus demographics plus antibodies place a patient into autoimmune hepatitis, primary biliary cholangitis, or primary sclerosing cholangitis within the first two sentences of the vignette. Autoimmune hepatitis is then the ANA and smooth-muscle-antibody interface hepatitis of the middle-aged woman with elevated IgG, confirmed on biopsy. The second half is treatment: steroid induction plus a steroid-sparing agent, the type one versus type two split, the simplified score, and why withdrawal is cautious because relapse is the rule.
Topics covered
Sorting framework: target cell sets the biochemical pattern
Demographics and IBD association across the three diseases
Antibody panel and IgG profile as the discriminator
Autoimmune hepatitis pathogenesis and two clinical faces
Type one versus type two serologic subtypes
Simplified scoring system and its blind spots
Interface hepatitis and ancillary histology
Steroid induction, azathioprine, and budesonide
Remission endpoints and cautious withdrawal
Key decisions
Sort the three immune liver diseases by pattern plus demographics plus antibodies: hepatocellular with ANA or smooth-muscle antibody and high IgG is autoimmune hepatitis, cholestatic with anti-mitochondrial antibody is primary biliary cholangitis, and cholestatic in a man with ulcerative colitis needing MRCP is primary sclerosing cholangitis.
Check a TPMT level before starting azathioprine, because TPMT-deficient patients metabolize it to toxic levels and develop severe myelosuppression; induce a non-cirrhotic adult with prednisone thirty to sixty milligrams daily plus azathioprine fifty to one hundred fifty milligrams daily.
Budesonide at nine milligrams daily is an alternative induction agent for non-cirrhotic patients but must not be used in cirrhosis, because portosystemic shunting bypasses first-pass metabolism, lowering efficacy and raising systemic exposure.
Treat acute severe autoimmune hepatitis with high-dose prednisone or IV methylprednisolone without azathioprine or budesonide, reassess every one to two weeks, and refer for transplant on failure to respond rather than escalating the dose.
Maintain biochemical remission (normal transaminases and normal IgG) for at least two years before considering withdrawal, ideally after a confirmatory biopsy, because most patients relapse within a few years of stopping.
Drug-induced autoimmune-like hepatitis from nitrofurantoin, minocycline, methyldopa, hydralazine, statins, or checkpoint inhibitors is the exception to lifelong therapy: it remits with drug withdrawal and a steroid taper, so the medication history is the discriminator.
In pregnancy continue both prednisone and azathioprine, because the risk of a flare on withdrawal exceeds the fetal risk, and postpartum flare is common.
For the full chapter with MCQs, tables, and primary-guideline references, visit www.boardpearls.com.
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(00:00) - The sorting framework and its variable
(00:30) - Target cell sets the biochemical pattern
(01:35) - Demographics, IBD, and the antibody panel
(04:08) - Autoimmune hepatitis and its two faces
(05:46) - The simplified scoring system
(06:36) - Interface hepatitis on biopsy
(07:22) - Induction and steroid-sparing maintenance
(09:02) - Remission and cautious withdrawal