Primary sclerosing cholangitis

Primary Sclerosing Cholangitis (PSC) is a chronic, progressive liver disease marked by inflammation and scarring of the bile ducts. These ducts carry digestive bile from the liver to the small intestine. Over time, inflammation causes the ducts to narrow and harden, leading to bile accumulation and liver damage. PSC is often associated with inflammatory bowel diseases, particularly ulcerative colitis.

Though rare, PSC can lead to serious complications like liver failure, repeated infections, and increased risk of liver cancer. There is currently no cure, but treatments can manage symptoms and slow progression.

Primary Sclerosing Cholangitis is a long-term liver condition where the bile ducts inside and outside the liver become inflamed, scarred, and eventually blocked. This disrupts bile flow, which is essential for digestion and the removal of toxins.

The disease develops gradually and may remain silent in early stages. As it progresses, it can cause liver damage, cirrhosis, and liver failure. PSC is considered an autoimmune disease, though the exact cause remains unknown.

Global Prevalence

PSC is a rare condition, affecting an estimated 1 in 10,000 people globally. It is more common in men than women and typically diagnosed between the ages of 30 and 50. It is also strongly linked to inflammatory bowel diseases, with up to 80% of PSC patients also having ulcerative colitis.

Prevalence in India

There is limited large-scale epidemiological data on PSC in India, but smaller studies suggest a growing number of diagnoses, likely due to better awareness and diagnostic capabilities. Estimates suggest that PSC may account for up to 2–5% of chronic liver disease cases in tertiary care settings.

• PSC can be categorised into two main types:
• Large-duct PSC: The more common type, involving the major bile ducts inside and outside the liver.
• Small-duct PSC: Involves only the smaller bile ducts within the liver. This form tends to have a better prognosis.

Other variations include:

• Overlap Syndromes: PSC can sometimes coexist with autoimmune hepatitis.
• PSC with IBD: Most commonly associated with ulcerative colitis but can also occur with Crohn’s disease.
   

The exact cause of PSC is unknown, but the following factors may contribute:

• Autoimmune Response: The immune system may mistakenly attack bile ducts.
• Genetics: A family history of PSC or autoimmune diseases increases risk.
• Inflammatory Bowel Disease (IBD): Particularly ulcerative colitis.
• Infections or Toxins: Exposure to certain environmental agents could be triggers.
• Gut Microbiome: Alterations in gut bacteria are being explored as a contributing factor.
   

Early stages of PSC may not present noticeable symptoms. As the disease progresses, common symptoms include:

• Persistent fatigue
• Itching (pruritus)
• Yellowing of the skin and eyes (jaundice)
• Abdominal discomfort, especially in the upper right quadrant
• Unexplained weight loss
• Dark urine and pale stools
• Episodes of fever and chills (due to bile duct infections)

Advanced stages may lead to signs of liver failure, including swelling of the abdomen (ascites), confusion (hepatic encephalopathy), and easy bruising.

Diagnosing PSC involves several steps:

• Medical History and Physical Exam: Evaluates symptoms and associated conditions.
• Blood Tests: Liver function tests, including ALP (alkaline phosphatase), are typically elevated.
• Imaging: MRCP (Magnetic Resonance Cholangiopancreatography) is the preferred imaging technique.
• Liver Biopsy: Used in uncertain cases or to assess the extent of liver damage.
• Endoscopic Retrograde Cholangiopancreatography (ERCP): Occasionally used for both diagnosis and treatment of strictures.

While there is no cure for PSC, treatments focus on managing symptoms and complications:

• Ursodeoxycholic Acid (UDCA): A bile acid used to improve liver enzyme levels. (Brand: Ursodol 30mg Tablet)
• Antibiotics: For treating infections of the bile ducts. (Brand: Cefix 400mg Tablet)
• Endoscopic Procedures: ERCP may relieve blockages by inserting stents.
• Liver Transplant: The only definitive treatment in cases of advanced liver failure.
• Symptom Management: Antihistamines for itching, and vitamin supplements for deficiencies.
   

Some individuals explore complementary therapies alongside standard care:

• Dietary Adjustments: Low-fat, nutrient-dense diet to support liver health.
• Herbal Remedies: Milk thistle is often cited, though evidence is limited.
• Probiotics: May help balance gut flora, especially in patients with IBD.
• Mind-Body Practices: Meditation, yoga, and acupuncture for symptom relief.

Always consult with a healthcare provider before trying alternative therapies.

• Male gender
• Age between 30–50 years
• Having ulcerative colitis or Crohn’s disease
• Family history of PSC or autoimmune diseases
• Genetic predisposition
   

If left untreated, PSC can lead to severe health issues:

• Liver Cirrhosis: Advanced liver scarring leading to failure.
• Cholangiocarcinoma: Bile duct cancer risk increases.
• Gallstones and Bile Duct Stones
• Repeated Infections: Cholangitis due to bile flow obstruction.
• Colon Cancer: Elevated risk in patients with associated ulcerative colitis.
• Vitamin Deficiencies: Fat-soluble vitamins (A, D, E, K) are poorly absorbed.

• Stay Active: Gentle exercise to maintain energy and muscle mass.
• Eat Well: Balanced diet with appropriate supplements.
• Routine Medical Care: Regular check-ups and tests.
• Monitor Symptoms: Report changes like worsening jaundice or fatigue.
• Mental Health: Join support groups or counselling.
• Avoid Alcohol: Essential to protect liver health.
   

• Myth: PSC always causes symptoms early.
   Fact: Many people remain asymptomatic for years.
• Myth: PSC is caused by alcohol use.
   Fact: PSC is not related to alcohol consumption.
• Myth: All PSC patients need a liver transplant.
   Fact: Not everyone progresses to liver failure.
   

Consult a healthcare provider if you experience:

• Persistent itching
• Yellowing of eyes or skin
• Fatigue that interferes with daily life
• Abdominal pain or swelling
• Unexplained weight loss
• Fever or chills indicating potential bile duct infection

• What stage is my liver disease?
• Do I need further imaging or biopsy?
• What treatment options do I have?
• Should I be screened for cancer?
• How often should I follow up?
• Are there clinical trials available?

• Offer Emotional Support: Be a good listener and show empathy.
• Help with Medical Appointments: Accompany them or help track test schedules.
• Encourage Healthy Habits: Promote good nutrition and activity.
• Educate Yourself: Learn about PSC to provide informed support.
• Join a Support Group: Both patients and caregivers benefit from shared experiences.
   

Primary Sclerosing Cholangitis is a challenging condition that demands long-term management. Early diagnosis, regular monitoring, and proactive symptom control can significantly improve quality of life. While the disease has no cure, advances in medical care and liver transplantation offer hope. Education, support, and a healthy lifestyle are key to coping with PSC effectively.