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Inflammation of Bile Ducts

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Primary sclerosing Cholangitis, PSC

  • The liver makes a chemical known as bile, which is necessary for the proper digestion of fat in our food. Bile enters tiny channels (bile ducts) in the liver for transport outside to the gallbladder, a sac used to store bile.
  • PSC describes a disease in which long term (chronic) inflammation (irritation, swelling, and damage) causes scarring and narrowing of the bile ducts inside and outside the liver. As a result, bile accumulates in the ducts and leaks into the liver, causing damage.

  • Some only find out after routine blood work shows abnormalities of bile duct chemicals known as enzymes (e.g., Alkaline phosphatase).
  • Fever
  • Chills
  • Itching
  • Jaundice -- yellowish tinge to the skin and the whites of the eyes
  • Fatigue
  • Diarrhea
  • Poor appetite and weight loss
  • Indigestion -- bloating (a feeling of excess gas in the abdomen) and problems with fatty foods
  • The symptoms are intermittent (on and off).
  • After 10-15 years, liver failure may occur.
  • Development of a cancer in the bile ducts known as Cholangiocarcinoma
  • There may be symptoms of associated conditions -- ulcerative colitisCrohn's Disease, and osteoporosis.

  • Unknown
  • A rare disease
  • Possibly immune system (body's natural defense system) involvement
  • No virus or other infectious agents
  • The process may lead to liver failure.
  • The liver is an important organ located in the right upper quadrant of the abdomen, and functions in the metabolism (making, storing, changing, using, and transforming to energy) of Proteins, fats and carbohydrates, destroying toxins (poisons), and breaking down drugs and other chemicals such as hormones.
  • Bile is important in digesting (breaking down and absorbing) fats and fat-soluble vitamins. It also helps in keeping the small intestine free of harmful bacteria.

  • History:
    1. Symptoms
    2. Illnesses -- gallstones, hepatitis, etc.
    3. Surgeries
    4. Habits -- alcohol, drugs, etc.
    5. Medications
    6. Allergies
    7. Family history
  • Medical exam:
    1. Fever
    2. Chills
    3. Fatty, greasy stool
    4. Jaundice
  • Blood tests may show:
    1. Increased white blood cells, Alkaline phosphatase (reflects obstruction of bile ducts), total Bilirubin (a bile pigment), and evidence of immune system attack (antineutrophil cytoplasmic antibody found in 70%).
    2. At times of suspected infection, Blood cultures (look for bacteria) are done.
    3. The doctor often consults with a gastroenterologist or GI specialist (digestive disease doctor).
    4. GI physician often does an ERCP, which is an X-Ray (radiograph or picture) test that involves injecting a dye into the bile ducts.
    5. A Liver biopsy is done by inserting a needlelike device into the liver, removing a tiny piece of tissue (containing the damaged ducts), and sending it to be examined under a microscope.

  • Men (75%) >women
  • Age -- symptoms start between 30-50 years of age
  • Associated with Inflammatory Bowel Disease -- 70% have ulcerative Colitis
  • Bacterial infections of the damaged bile ducts occur and give rise to some of the symptoms (fever, chills).

  • Cholestyramine, Benadryl, or Rifampin for itching
  • Antibiotics help at times of infection
  • Corticosteroids -- may help in suppressing the immune attack.
  • Vitamins are given (K, A, and D) to replace the ones lost because of fatty stool.
  • Stenting (placing a flexible tube) or balloon dilation (enlarging) of the narrowed ducts is done in some cases.
  • Liver transplantation (from a donor) is done if the liver damage is severe (Cirrhosis).

  • Contact your doctor if you have sudden high fever and chills, appear jaundiced, and you vomit blood.




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