Primary Sclerosing Cholangitis (PSC)
Why in news?
Recent developments in Primary Sclerosing Cholangitis (PSC) treatment highlight promising results from a new monoclonal antibody called nebokitug. Phase 2 trials show nebokitug reduces liver stiffness and fibrosis biomarkers safely.​
About PSC
- Primary Sclerosing Cholangitis (PSC) is a chronic liver disease characterized by inflammation, fibrosis, and scarring of the bile ducts inside and outside the liver, leading to bile flow obstruction.
- This progressive condition often results in cholestasis, cirrhosis, and eventual liver failure if untreated.
- Liver transplantation remains the only definitive cure.​
Symptoms
- Many patients experience no early symptoms, with diagnosis prompted by abnormal liver tests.
- Common signs include fatigue, intense itching (pruritus), jaundice, abdominal pain, and enlarged liver or spleen.
- Advanced cases may involve fat-soluble vitamin deficiencies, gallstones, or bacterial cholangitis.​
Causes and Risk Factors
- The exact cause is unknown but involves genetic susceptibility, immune dysregulation, and possible environmental triggers like gut bacteria.
- PSC strongly associates with inflammatory bowel disease, especially ulcerative colitis (up to 70-80% of cases).
- It predominantly affects men aged 30-40.​
Diagnosis
- Elevated alkaline phosphatase and gamma-glutamyl transferase indicate cholestasis. Imaging like MRCP or ERCP reveals characteristic "beaded" bile duct strictures.
- Liver biopsy may confirm fibrosis but is not always needed.​
Treatment and Prognosis
- No medical therapy halts progression; ursodeoxycholic acid manages symptoms but lacks proven disease-modifying effects.
- Endoscopic interventions treat strictures, while complications like cholangiocarcinoma (10-20% risk) require surveillance.
- Median survival without transplant is about 10 years.​
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