• Clinical science

Primary biliary cholangitis

Abstract

Primary biliary cholangitis (PBC; also known as primary biliary cirrhosis) is a chronic progressive liver disease of autoimmune origin that is characterized by destruction of the intralobular bile ducts. The pathogenesis of PBC is unclear; however, it primarily affects middle-aged women and is frequently associated with other autoimmune conditions. In the early stages, PBC is typically asymptomatic. Fatigue is the most common initial complaint. In advanced disease, increased fibrotic changes lead to typical signs of cholestasis (e.g., jaundice) and portal hypertension (e.g., ascites, gastrointestinal bleeding). Elevated cholestasis parameters (ALP, γ-GT, bilirubin) as well as antimitochondrial antibodies (AMAs) help establish the diagnosis. Management consists of slowing disease progression with ursodeoxycholic acid and relieving symptoms. Liver transplantation is the only definitive treatment.

Epidemiology

References:[1][2]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • Idiopathic: however, generally accepted as an autoimmune disease
  • Associated with other autoimmune conditions (see “Clinical features” below)
  • Positive family history is a predisposing factor.

References:[3][1]

Pathophysiology

  • Inflammation and progressive destruction (likely due to an autoimmune reaction) of the small and medium-sized intrahepatic bile ducts (progressive ductopenia) → defective bile duct regenerationchronic cholestasis → secondary hepatocyte damage due to increased concentration of toxins that typically get excreted via bile → gradual portal and periportal fibrotic changes → liver failure → liver cirrhosis and portal hypertension (in advanced stage)

References:[3]

Clinical features

Patients are initially often asymptomatic (25% of cases are diagnosed incidentally). Signs and symptoms are mainly due to the resulting cholestasis, liver cirrhosis, and portal hypertension.

References:[3][1]

Diagnostics

  • Laboratory tests
  • Liver biopsy
    • Can confirm the diagnosis (although not required)
    • Necessary for staging

References:[1]

Pathology

Histopathological stages

Differential diagnoses

The differential diagnoses listed here are not exhaustive.

Treatment

There is no cure for PBC. Treatment consists of slowing disease progression and alleviating symptoms. Liver transplantation is the only definitive treatment.

References:[3]

Complications

References:[1]

We list the most important complications. The selection is not exhaustive.