Primary Sclerosing Cholangitis (PSC): Definition, Causes, Risk Factors, Symptoms, Diagnosis, Treatment, Complications, Home Remedies

Primary Sclerosing Cholangitis (PSC) is a medical illness of the liver caused by inflammation and hardening of bile duct in the liver. PSC is a progressive medical condition. Individual suffering from Primary Sclerosing Cholangitis usually ends up with liver failure requiring liver transplant. In this article, we will discuss in detail, the various causes, symptoms, and treatments for Primary Sclerosing Cholangitis.

• Primary Sclerosing Cholangitis (PSC)-
  • Medical disorder of the bile duct results in inflammation of bile ducts followed by scarring.¹
  • Cholangitis is a medical term used to express the inflammation of bile duct.
  • Sclerosing means hardening or scarring of bile ducts due to chronic inflammation.

• Bile Duct Obstruction-
  • Primary sclerosing cholangitis (PSC) is a disease of the bile ducts that causes inflammation and scarring.
  • Scarring causes subsequent obstruction of bile ducts both at an intrahepatic (inside the liver) and extrahepatic (outside the liver) level.²

• Progressive Disease-
  • Primary Sclerosing Cholangitis is a progressive medical condition, which leads to liver damage and ultimately liver failure.³

• Liver Failure-
  • Bile is secreted by liver and stored in gall bladder. Bile is carried to gall bladder through bile duct. Scarring of bile duct causes accumulation of bile in bile duct. Bile spills in to liver cells and damages liver cells resulting in cirrhosis of liver. ⁴
  • Cirrhosis of liver is irreversible change of the liver cells resulting in non-functioning liver cells and liver failure.⁵
  • The progression of the disease ends in liver failure or cirrhosis of the liver.
  • The inflammation impedes the flow of bile to the gut, which can ultimately lead to cirrhosis of the liver, liver failure and liver cancer.
  • At present, liver transplant is the only known treatment for Primary Sclerosing Cholangitis, but it is considered to be the last resort in cases where liver is severely damaged.⁶

• Progression of Primary Sclerosing Cholangitis (PSC) –
  • Slow
  • Symptoms may remain stable for several weeks to months.
  • Liver failure may occur after 15 to 20 years.

• Cause is Unknown
  • Definitive cause is not found
  • Immunosuppressant not effective⁵
• Autoimmune Disease
  • Associated with inflammatory bowel disease
  • Histopathological findings of autoimmune hepatitis and primary sclerosing cholangitis were similar. ⁷
  • Lab studies has shown majority of cases had autoreactive IgA antibodies.^{8, 9}
• Viral Infection
  • Associated with Viral hepatitis
• Genetic Malfunction

• Age: Primary Sclerosing Cholangitis can occur at any age but it is usually seen in individuals between the ages of 30 to 50.
• Sex: Primary Sclerosing Cholangitis is seen to occur more in males than females.
• Inflammatory Bowel Disease: A majority of people affected with Inflammatory Bowel Disease end up having Primary Sclerosing Cholangitis. The connection between these two diseases is still not established
• Ulcerative Colitis- Disease is associated with ulcerative colitis.

• Weakness
• Fatigue
• Muscle ache
• Fever with chills
• Loss of weight

• Pain in the abdomen
• Diarrhea
• Severe itching
• Itching/Pruritus
• Jaundice
• Malabsorption syndrome
• Fatty stool
• Hepatomegaly- enlarge liver
• Esophageal avarices- causes bleeding
• Dark urine
• Hepatic encephalopathy- confusion or coma.

• Complete Blood Examination
  • Low hemoglobin
• Liver Function Test-
  • Increase blood bilirubin
  • Increase liver enzymes
• Renal Function Test-
  • Blood Urea Nitrogen- elevated
  • Serum Creatinine- elevated
• Electrolytes-
  • Normal findings

• X-rays of Bile Ducts-
  • Enlarge liver
  • Rule out any radio-opaque shadow.
• MRI of Bile Ducts-
  • This again can confirm whether there is any type of scarring or inflammation of the bile ducts

• Liver biopsy can be done to find out the degree of liver damage

• Examination indicates breading of intrahepatic and extra-hepatic bile duct.

• Observed in 20% to 50% of patient diagnosed of Primary Sclerosing Cholangitis.

• Treatment for Pruritus-
  • Cholestyramine- Binds with bile in intestine and prevents bile absorption back into blood.
  • Anti-histaminic- Benadryl prevents or reduces itching.
• Treatment of Infection-
  • Antibiotics- Frequent bacterial infections are treated with antibiotics.
• Vitamin Deficiency-
  • Vitamin deficiency is often observed with liver cirrhosis.
  • Supplement vitamin A, D, E and K
• Corticosteroids-
  • Corticosteroid is used as an immunosuppressant.
  • Treatment helps to reduce symptoms and also severity of symptoms.
  • Corticosteroid also helps to reduced pruritus or itching.
• Immunosuppressant-
  • Immunosuppressant like cyclophosphamide are prescribed with corticosteroids.
• Ursodeoxycholic Acid
  • Improves liver function test when prescribed following ERCP and dilatation of bile duct
  • Some studies have shown no improvement in symptoms of pruritus and fatigue.
  • Patients were in need of liver transplantation in spite of improvement in blood test.
  • Percentage of incidence of death was same as patient who was not treated.
• ERCP-Endoscopic Retrograde Cholangiopancreatogram
  • Procedure is performed to dilate common bile duct.
  • Bile duct dilatation may help to improve the bile flow.
• Liver Transplantation-Indications for liver transplant surgery:
  • Recurrent infection not responding to antibiotic treatment,
  • Portal hypertension
  • Severe liver function failure.

• Liver failure- Cirrhosis of liver
• Frequent infections
• Portal hypertension- causes profuse esophageal bleeding
• Thinning of bones
• Cancer of the bile duct
• Cancer of the colon

If an individual has been diagnosed with Primary Sclerosing Cholangitis (PSC) then that individual can take the following steps to cut down the symptoms he or she might experience:

• Abstaining from alcohol.
• Consider Hepatitis A and B vaccinations.
• Use chemicals with caution whether at home or work.
• Abstain from smoking.
• Follow a healthy diet.
• Have adequate weight control.

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