Liver & intrahepatic bile ducts

Biliary tract disease

Autoimmune cholangitis

Editor-in-Chief: Debra L. Zynger, M.D.
Raul S. Gonzalez, M.D.

Last author update: 10 December 2020
Last staff update: 14 July 2022

Copyright: 2002-2024,, Inc.

PubMed Search: Autoimmune cholangitis liver

Raul S. Gonzalez, M.D.
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Cite this page: Gonzalez RS. Autoimmune cholangitis. website. Accessed April 14th, 2024.
Definition / general
Essential features
  • Mimics primary biliary cholangitis clinically and histologically, except antimitochondrial antibody is not detected
  • Advanced techniques often do uncover antimitochondrial antibody in these patients
  • Also called antimitochondrial antibody negative primary biliary cholangitis (AMA negative PBC), autoimmune cholangiopathy
ICD coding
  • ICD-10: K74.3 - primary biliary cirrhosis
  • Represents 5 - 10% of patients who otherwise fit diagnostic criteria for primary biliary cholangitis
  • Not applicable
  • Unclear; presumably autoimmune
Clinical features
  • More common in women; causes pruritus, fatigue
  • Patients may be slightly younger on average than those with primary biliary cholangitis (Dig Dis Sci 1995;40:1232)
  • May have worse outcome than antimitochondrial antibody positive primary biliary cholangitis patients (Scand J Gastroenterol 2016;51:745)
  • Some patients may seroconvert to antimitochondrial antibody positivity (J Hepatol 1996;25:574)
  • Positive antinuclear antibody and smooth muscle antibody (at higher rates than primary biliary cholangitis patients); negative antimitochondrial antibody
  • May have higher aspartate transaminase and bilirubin levels than primary biliary cholangitis patients (Hepatology 2000;31:1231)
  • More sophisticated techniques (including primary biliary cholangitis specific antinuclear antibodies, such as Gp210 and sp100) have shown that many antimitochondrial antibody negative primary biliary cholangitis patients are in fact antimitochondrial antibody positive (Clin Res Hepatol Gastroenterol 2016;40:553, Hepatology 2007;45:659)
  • Emerging biomarkers for diagnosis have been identified, though they show poor sensitivity (Clin Chem Lab Med 2017;56:25, Gastroenterol Res Pract 2019;2019:8959103)
Case reports
  • Ursodeoxycholic acid, which may be less successful than for antimitochondrial antibody positive patients
Microscopic (histologic) description
  • Essentially the same as primary biliary cholangitis, except perhaps with less chronic portal inflammation
  • Florid duct lesions (granulomas destroying bile ducts), lobular granulomas, duct injury and disarray, duct inflammation, ductular reaction, lobular cholestasis, ductopenia late
Microscopic (histologic) images

Contributed by Raul S. Gonzalez, M.D.

Portal tract inflammation

Bile duct injury

Focal cholestasis

Florid duct lesion

Sample pathology report
  • Liver, biopsy:
    • Biliary pattern injury with duct injury, ductular reaction and focal florid duct lesions (see comment)
    • Comment: The changes are compatible with primary biliary cholangitis. Given the patient's reported negative antimitochondrial antibody, the overall findings may be most consistent with autoimmune cholangitis (antimitochondrial antibody negative primary biliary cholangitis).
Differential diagnosis
Board review style question #1
What is the key distinction between primary biliary cholangitis and autoimmune cholangitis?

  1. Alkaline phosphatase level
  2. Antimitochondrial antibody status
  3. Antineutrophil cytoplasmic antibody status
  4. Antinuclear antibody status
  5. Anti-smooth muscle antibody status
Board review style answer #1
B. Antimitochondrial antibody status

Comment Here

Reference: Autoimmune cholangitis
Board review style question #2

A 45 year old woman complaining of jaundice is found to have elevated liver enzymes. The figure above shows a liver biopsy. The patient's physician orders serology for antimitochondrial antibodies, which returns as negative. Which of the following is the most likely explanation?

  1. The liver sample must have actually come from a different patient
  2. The patient has autoimmune cholangitis
  3. The patient has sarcoidosis
  4. The patient has tuberculosis
  5. The serology testing returned a false negative result
Board review style answer #2
B. The patient has autoimmune cholangitis

Comment Here

Reference: Autoimmune cholangitis
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