Primary Sclerosing Cholangitis (PSC)

Primary sclerosing cholangitis (PSC) is a rare and chronic liver disease characterized by inflammation and scarring of the bile ducts, which are the tubes that carry bile from the liver to the gallbladder and small intestine. This condition leads to narrowing and constriction of the bile ducts, resulting in the accumulation of bile in the liver and subsequent liver damage.

Causes and Risk Factors

The exact cause of PSC is unknown, but it is believed to be an autoimmune disease, where the immune system mistakenly attacks the bile ducts. Several risk factors have been identified, including:

• Inflammatory bowel disease (IBD), such as ulcerative colitis or Crohn's disease
• Family history of PSC
• Northern European ancestry
• Male sex

Symptoms

The symptoms of PSC may vary from person to person and can include:

• Fatigue
• Weight loss
• Abdominal pain
• J jaundice (yellowing of the skin and eyes)
• Itching (pruritus)
• Dark urine
• Pale or clay-colored stools

Diagnosis

The diagnosis of PSC is typically made using a combination of the following tests:

• Endoscopic retrograde cholangiopancreatography (ERCP)
• Magnetic resonance cholangiopancreatography (MRCP)
• Computed tomography (CT) scan
• Liver biopsy
• Blood tests to check liver function and inflammatory markers

Treatment

The treatment of PSC is focused on managing symptoms, slowing disease progression, and preventing complications. Treatment options may include:

• Ursodeoxycholic acid (UDCA) to improve bile flow and reduce liver inflammation
• Corticosteroids to reduce inflammation
• Immunosuppressive medications to suppress the immune system
• Endoscopic therapy to dilate narrowed bile ducts
• Surgical intervention, such as liver transplantation, in advanced cases

Complications

PSC can lead to several complications, including:

• Liver cirrhosis and failure
• Bile duct cancer (cholangiocarcinoma)
• Gallstones
• Osteoporosis
• Fat-soluble vitamin deficiencies

Prognosis

The prognosis for PSC varies depending on the severity of the disease and the effectiveness of treatment. With proper management, many people with PSC can lead active lives, but the condition can progress to liver failure and require transplantation in some cases.