Variant and Specific Forms of Autoimmune Cholestatic Liver Diseases.
Georgios N DalekosNikolaos K GatselisPublished in: Archivum immunologiae et therapiae experimentalis (2019)
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the main autoimmune cholestatic liver diseases. IgG4-associated sclerosing cholangitis is another distinct immune-mediated cholestatic disorder of unknown aetiology that is frequently associated with autoimmune pancreatitis or other IgG4-related diseases. Although the majority of PBC and PSC patients have a typical presentation, there are common and uncommon important variants or specific subgroups that observed in everyday routine clinical practice. In this updated review, we summarize the published data giving also our own experience on the variants and specific groups of autoimmune cholestatic liver diseases. Actually, we give in detail the underlining difficulties and the rising dilemmas concerning the diagnosis and management of these special conditions in the clinical spectrum of autoimmune cholestatic liver diseases including the IgG4-associated sclerosing cholangitis highlighting also the uncertainties and the potential new eras of the research agenda.
Keyphrases
- liver injury
- drug induced
- multiple sclerosis
- liver fibrosis
- clinical practice
- end stage renal disease
- copy number
- newly diagnosed
- ulcerative colitis
- chronic kidney disease
- systematic review
- ejection fraction
- prognostic factors
- gene expression
- machine learning
- big data
- risk assessment
- electronic health record
- case report
- patient reported outcomes
- artificial intelligence