Autoimmune cholangitis

Autoimmune cholangitis (AIC), also known as autoimmune cholangiopathy, is a chronic inflammation of the liver and a variant syndrome of autoimmune hepatitis.9

Autoimmune cholangitis is an idiopathic disorder with mixed hepatocellular and biliary involvement.10
Clinical features typically include fatigue and pruritus with laboratory indications of cholestasis. Serological findings include high titres of antinuclear antibodies (ANA) with or without smooth muscle antibodies (SMA) and an absence of antimitochondrial antibodies (AMA).9-11 Progression to sclerosing cholangitis is possible.12-16
Diagnosis
There are no uniform diagnostic criteria for autoimmune cholangitis and its characterisation is still evolving; however diagnostic criteria include:10
  • Absence of other aetiologic bases for liver injury
  • Laboratory features of immunoreactivity, including ANA and/or SMA seropositivity and/or hypergammaglobulinaemia
Histopathology of liver tissue reveals bile duct injury in the form of cholangitis and ductopenia with signs of variable intensity of autoimmune hepatitis.9
Back to diagnostic algorithm

9. Sharma B, Raina S, Sharma R. Autoimmune cholangitis: a variant syndrome of autoimmune hepatitis. Case Reports Hepatol 2014;2014:501530.

10. Czaja AJ, Carpenter HA, Santrach PJ, Moore SB. Autoimmune cholangitis within the spectrum of autoimmune liver disease. Hepatology 2000;31:1231-8.

11. Ben-Ari Z, Czaja AJ. Autoimmune hepatitis and its variant syndromes. Gut 2001;49:589-94.
12. Terziroli Beretta-Piccoli B, Vergani D, Mieli-Vergani G. Autoimmune sclerosing cholangitis: Evidence and open questions. J Autoimmun 2018;95:15-25.

13. Mieli-Vergani G, Vergani D, Baumann U, et al. Diagnosis and management of pediatric autoimmune liver disease: ESPGHAN Hepatology Committee position statement. J Pediatr Gastroenterol Nutr 2018;66:345-60.

14. Adike A, Carey EJ, Lindor KD. Primary sclerosing cholangitis in children versus adults: lessons for the clinic. Expert Rev Gastroenterol Hepatol 2018;12:1025-32.

15. Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999;31:929-38.

16. Mieli-Vergani G, Vergani D. Sclerosing cholangitis in children and adolescents. Clin Liver Dis 2016;20:99-111.