From the Guidelines
Lymphocytic cholangiopathy is a condition characterized by inflammation of the bile ducts with lymphocyte infiltration, and its treatment involves immunosuppressive therapy, with corticosteroids as the initial treatment of choice, and azathioprine considered in certain cases. This condition is associated with autoimmune features, and its management is crucial to prevent disease progression and complications. According to the EASL clinical practice guidelines 1, immunosuppressive treatment has been shown to exert a marked effect on inflammatory activity, and complete long-term remission after three months of treatment has been reported.
Key Features of Lymphocytic Cholangiopathy
- Inflammation of the bile ducts with lymphocyte infiltration
- Autoimmune features
- May involve proximal extrahepatic and intrahepatic bile ducts
- Prone to relapse after stop of treatment, especially in patients with alterations of proximal extrahepatic and intrahepatic bile ducts
Treatment Approach
- Corticosteroids as the initial treatment of choice
- Azathioprine at doses up to 2 mg/kg/d considered in patients with proximal and intrahepatic stenoses, and those after relapse during/after corticosteroid therapy
- Treatment duration of 3 months may be sufficient for some patients, but long-term maintenance therapy at low doses may be required when disease activity has not completely come to a standstill or has relapsed, as reported in the EASL clinical practice guidelines 1.
Monitoring and Follow-up
- Regular monitoring of liver function tests every 3-6 months to assess treatment response
- Patients should be monitored for complications and referred for liver transplantation evaluation when appropriate, considering the potential progression to cirrhosis and liver failure.
From the Research
Definition and Characteristics of Lymphocytic Cholangiopathy
- Lymphocytic cholangiopathy is characterized by inflammation of the bile ducts with lymphocyte infiltration 2
- It is a form of chronic granulomatous lymphocytic cholangitis of the small bile ducts, which is a key feature of primary biliary cholangitis (PBC) 2
- The pathogenesis of PBC involves inflammatory damage of bile duct epithelium secondary to innate and adaptive immune responses, and toxicity from accumulated bile acids 2
Related Conditions and Treatment
- Primary biliary cholangitis (PBC) is an autoimmune liver disease that can lead to cirrhosis and extrahepatic complications such as dyslipidemia, metabolic bone disease, and fat-soluble vitamin deficiency 2
- Ursodeoxycholic acid (UDCA) is a well-established therapy for PBC, and combination therapy with glucocorticoids and immunosuppressants may improve long-term survival rates in PBC patients 3
- UDCA has also been used in the treatment of drug-induced liver injury (DILI), and has been shown to have hepatoprotective and anti-cholestatic mechanisms 4
Clinical Presentation and Diagnosis
- The clinical presentation of patients with lymphocytic cholangiopathy may include symptoms such as fever, abdominal complaints, and increased cholestasis on liver function tests 5
- Diagnosis is typically made by imaging studies of the bile ducts, such as endoscopic retrograde cholangiopancreaticography (ERCP) or percutaneous transhepatic cholangiodrainage (PTCD) 5