Published online Oct 8, 2016. doi: 10.4254/wjh.v8.i28.1157
Peer-review started: March 7, 2016
First decision: April 15, 2016
Revised: July 22, 2016
Accepted: August 6, 2016
Article in press: August 8, 2016
Published online: October 8, 2016
Primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) constitute the classic autoimmune liver diseases (AILDs). While AIH target the hepatocytes, in PBC and PSC the targets of the autoimmune attack are the biliary epithelial cells. Persistent liver injury, associated with chronic AILD, leads to un-resolving inflammation, cell proliferation and the deposition of extracellular matrix proteins by hepatic stellate cells and portal myofibroblasts. Liver cirrhosis, and the resultant loss of normal liver function, inevitably ensues. Patients with cirrhosis have higher risks or morbidity and mortality, and that in the decompensated phase, complications of portal hypertension and/or liver dysfunction lead to rapid deterioration. Accurate diagnosis and monitoring of cirrhosis is, therefore of upmost importance. Liver biopsy is currently the gold standard technique, but highly promising non-invasive methodology is under development. Liver transplantation (LT) is an effective therapeutic option for the management of end-stage liver disease secondary to AIH, PBC and PSC. LT is indicated for AILD patients who have progressed to end-stage chronic liver disease or developed intractable symptoms or hepatic malignancy; in addition, LT may also be indicated for patients presenting with acute liver disease due to AIH who do not respond to steroids.
Core tip: In chronic liver disease, including autoimmune liver diseases, perpetual liver injury leads to persistent inflammation, cell proliferation and the deposition of extracellular matrix proteins. If left untreated, this process eventually leads to the development of liver cirrhosis, characterised by the presence of fibrosis and nodular regeneration. Liver biopsy is currently the gold standard technique, but highly promising non-invasive methodology is under development.