From the Guidelines
Cholestasis is primarily caused by intrahepatic or extrahepatic bile duct obstruction, with underlying causes including primary biliary cirrhosis, primary sclerosing cholangitis, drug-induced cholangiopathy, and genetic disorders such as ABCB4 deficiency, as outlined in the most recent guidelines 1. The causes of cholestasis can be broadly categorized into intrahepatic and extrahepatic causes. Intrahepatic causes include:
- Hepatocellular cholestasis due to sepsis, viral hepatitis, alcoholic or non-alcoholic steatohepatitis, drug-induced cholestasis, and genetic disorders such as BRIC, PFIC, and ABCB4 deficiency 1
- Cholangiocellular cholestasis due to primary biliary cirrhosis, primary sclerosing cholangitis, IgG4-associated cholangitis, and idiopathic adulthood ductopenia 1 Extrahepatic causes include:
- Bile duct obstruction due to stones, tumors, cysts, or strictures 1
- Other causes such as vascular disorders, cirrhosis, and nodular regenerative hyperplasia 1 It is essential to identify the underlying cause of cholestasis to provide appropriate treatment and management, as outlined in the recent guidelines 1. Treatment of cholestasis depends on the underlying cause and may involve medications such as ursodeoxycholic acid, cholestyramine, and antihistamines, as well as procedures like ERCP or surgery for extrahepatic obstruction 1. Regular monitoring of liver function tests and imaging studies is crucial to track disease progression and adjust treatment accordingly 1. Prompt diagnosis and management of cholestasis are critical to prevent complications like liver fibrosis or cirrhosis, as emphasized in the recent guidelines 1.
From the Research
Causes of Cholestasis
The causes of cholestasis can be categorized into intrahepatic and extrahepatic causes.
- Intrahepatic cholestasis is caused by conditions that affect the liver cells or the small bile ducts within the liver, such as primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) 2, 3.
- Extrahepatic cholestasis is caused by conditions that affect the large bile ducts outside the liver, such as bile duct obstruction or bile duct cancer.
Specific Causes of Cholestasis
Some specific causes of cholestasis include:
- Drug-induced cholestatic disease, which is more common in the elderly 4.
- Cholestatic viral hepatitis, which is more common in young people with risk factors 4.
- Cholestasis of pregnancy, which occurs in the third trimester of gestation 4.
- Benign recurrent intrahepatic cholestasis, which is characterized by a familial history of recurrent cholestasis 4.
- Alcoholic hepatitis, which is characterized by the presence of vascular spider nevi, ascites, and a history of alcohol abuse 4.
- Neonatal cholestasis syndromes, which include conditions such as CMV, toxoplasma, and rubella infections, as well as metabolic defects like cystic fibrosis and bile acid synthesis defects 4.
Chronic Cholestatic Liver Diseases
Chronic cholestatic liver diseases, such as PBC and PSC, are common causes of chronic cholestasis 2, 3.
- PBC is characterized by cholestatic laboratory features, antimicrobial antibody positivity, and typical histological patterns 2.
- PSC is characterized by progressive inflammatory and fibrosing impairment of the intrahepatic and extrahepatic biliary ducts, and is often associated with inflammatory bowel disease 2.