Primary Biliary Cholangitis

#med #disease #y2023

Presentation :

Liver chemistry tests usually demonstrate a cholestatic pattern of injury, and the alkaline phosphatase may be significantly elevated.
Diagnosis of PBC does not require liver biopsy when the ALP level is at least 1.5 times the upper limit of normal and antimitochondrial antibody results are positive or, if results are negative, other PBC-specific autoantibodies, including sp100 or gp210, are present.
- In patients with negative antibody results and strong suspicion for PBC, liver biopsy is necessary
Elastography can be used for fibrosis staging.
Screening:
- Bone mineral density should be assessed every 2 years, and bisphosphonate therapy should be considered if the result is in the osteoporotic range
- PBC is associated with other autoimmune conditions, particularly autoimmune thyroid disease. Thus, TSH should be checked yearly.
- In patients with cirrhosis and a PBC score of 4.1 or greater or transient elastography results of 17 kPa or greater, upper endoscopy is indicated to assess for esophageal varices.
- Men with PBC and all patients with PBC and cirrhosis should be screened for hepatocellular carcinoma
- First-degree relatives of patients with PBC, especially women, should be screened by periodically checking ALP level

Initial treatment is ursodeoxycholic acid, which results in histologic improvement, improves survival rates, and diminishes the need for liver transplantation.
- Response to treatment is defined by improvement of the ALP level to less than 1.67 times the upper limit of normal.
- If disease does not respond should receive fibrates (off label) or obeticholic acid.

Patients who present with normal bilirubin and albumin levels and respond to treatment have a life expectancy similar to that of individuals without PBC.
Liver transplant outcomes for patients with PBC are excellent, with a 1-year survival rate greater than 90% and a recurrence rate of approximately 20% at 5 years after liver transplantation