Editing Primary biliary cholangitis

Previously called 'primary biliary cirrhosis'

== Aetiology ==

* Unknown, but probably an environmental trigger in a genetically-susceptible person
* Environmental
** 25 per million in Australia; 250 per million in UK
** Infections possibly causing molecular mimicry - viruses, bacteria
** Chemical
* Genetic
** Clustering within families

== Pathophysiology ==

* T-lymphocyte-mediated attack on small intralobular bile duct epithelial cells
* Leads to gradual destruction and eventual disappearance, causing cholestasis
* Eventual result is cirrhosis and liver failure
* Anti-mitochondrial antibodies are the serological hallmark
== Presentation ==

* Asymptomatic
* Symptomatic
** Fatigue
** Pruritis
* Associated autoimmune disorders

== Diagnosis ==

* Suspect in patients with elevated ALP without anatomical obstruction; especially with previous autoimmune disease
* Criteria
** ALP >1.5x ULN
** AMA at a titre of 1:40 or higher
** Histological evidence of PBC

== Management ==

* Liver protection
** Immunise against hepatitis
** Avoid alcohol
* Medications
** Ursodeoxycholic acid - slows progression, improves LFTs
* Liver transplant can be considered
* Look for and treat complications of cholestasis

== Complications ==

* Cirrhosis
* HCC
* Metabolic bone disease

== Prognosis ==

* Majority now have normal life expectancies

[[Category:Biliary]]