Primary Sclerosing Cholangitis
Chronic progressive disease causing inflammation and fibrosis of bile ducts.
Overview
Primary sclerosing cholangitis (PSC) is a chronic disease in which the bile ducts become inflamed and scarred, causing progressive narrowing and blockage of bile flow. Over time this can lead to liver damage, recurrent infections, and an increased risk of bile duct and colon cancer. PSC is strongly associated with inflammatory bowel disease. Management is specialised and often multidisciplinary.
Causes
- Autoimmune process (immune system attacks the bile ducts)
- Genetic predisposition
- Strong association with ulcerative colitis and Crohn's disease
Risk Factors
- Inflammatory bowel disease
- Male gender
- Age 30–50
- Family history of autoimmune disease
Symptoms
Diagnosis
- MRCP (characteristic beaded appearance of bile ducts)
- Liver function tests
- Autoimmune blood markers
- ERCP (for dominant strictures)
- Liver biopsy in selected cases
- Colonoscopy (to assess associated IBD)
Treatment Options
Surgical Treatment
There is no cure short of liver transplantation, but management focuses on relieving obstruction and complications. Dominant strictures are treated endoscopically with dilatation or stenting. Dr. Bhagat manages the surgical and biliary aspects, including drainage procedures and coordination for liver transplantation in advanced disease. Ongoing surveillance for bile duct and colon cancer is essential.
Recovery
PSC is a chronic, progressive condition managed over years. Endoscopic treatments provide symptom relief. For advanced liver disease, transplantation offers the best long-term outcome. Regular monitoring is key to detecting complications early.
Prevention
- No known prevention
- Regular surveillance for cancer (bile duct and colon)
- Management of associated inflammatory bowel disease
- Prompt treatment of biliary infections
Frequently Asked Questions
Is there a cure for PSC?
Why is cancer screening important in PSC?
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