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Primary Sclerosing Cholangitis

Primary sclerosing cholangitis is a disease of the bile ducts both inside and outside of your liver. "Cholangitis" refers to inflamed bile ducts, while "sclerosing" describes the hardening and scarring of the bile ducts that result from chronic inflammation. Scarred bile ducts can impede the passage of bile through the ducts, compromising your liver's ability to function properly.

Abdominal pain, itchiness, yellowing of your eyes and skin, fevers and fatigue are typical signs and symptoms of bile blockages that may be due to primary sclerosing cholangitis. It's not clear what causes primary sclerosing cholangitis, although it may develop due to an autoimmune disorder. Primary sclerosing cholangitis is often associated with inflammatory bowel disease.

Primary sclerosing cholangitis usually progresses slowly but invariably leads to liver disease. Medical and surgical treatments may improve the symptoms, but a liver transplant is the only cure for this condition.

Your liver is an indispensable, multifunctional organ in your body. Located on the upper right side of your abdomen, this large organ metabolizes carbohydrates and fats and removes wastes from your bloodstream. Your liver also produces bile, a greenish fluid that carries away waste and facilitates food digestion in your small intestine. After being secreted by the liver cells, bile flows through numerous tiny ducts throughout the liver and joins a larger bile duct called the hepatic duct, much like many small streams converge to form a river. The hepatic duct
joins the larger common bile duct, which empties into the top portion of the small intestine (duodenum).

When the bile ducts inside and outside of your liver become inflamed, they can scar and narrow, interrupting the flow of bile. Bile that's stalled in the bile ducts can enter your bloodstream, causing your skin to develop a yellowish hue (jaundice). Bile also injures liver cells — which eventually can irreversibly damage your liver.

It's not clear what causes primary sclerosing cholangitis. It's believed that the condition may be an autoimmune disorder — perhaps precipitated by a bacterial or viral infection or exposure to a toxin — in people who have a genetic predisposition to develop the disease.

Primary sclerosis cholangitis and inflammatory bowel disease
Primary sclerosing cholangitis is strongly associated with inflammatory bowel disease (Crohn's disease and ulcerative colitis). Inflammatory bowel disease is characterized by chronic inflammation of your digestive tract.

Up to 80 percent of people with primary sclerosing cholangitis have or will develop inflammatory bowel disease, but a much smaller percentage (about 5 percent) of people initially diagnosed with inflammatory bowel disease go on to develop primary sclerosing cholangitis.

Risk Factor :
Anyone can develop primary sclerosing cholangitis, but several factors may raise your risk of developing this condition :

  • Age. Primary sclerosing cholangitis most often affects people in their 30s and 40s, but can occur at any age.
  • Sex. Men are twice as likely as women are to develop primary sclerosing cholangitis.
  • Family history. Primary sclerosing cholangitis tends to run in families. Mutations in certain genes can increase your risk of developing primary sclerosing cholangitis.

When to seek medical advice :
Call your doctor if you experience any signs or symptoms of liver disease, including :

  • Abdominal bloating or pain
  • Yellow discoloration of your skin or eyes
  • Abnormal-looking stools, including stools that are pale, bloody or tar-like
  • Dark yellow or brown urine

If you have primary sclerosing cholangitis, it's important to let your doctor know if you notice either of the following :

  • Bothersome symptoms, such as itching, persistent jaundice, or swelling of your abdomen or feet and ankles
  • Symptoms that aren't relieved by the medications that you're taking

If you have chronic inflammatory bowel disease, be sure to see your doctor regularly because routine blood tests may detect primary sclerosing cholangitis before it becomes symptomatic.

Signs and symptoms of primary sclerosing cholangitis develop when normal liver function is hindered by inflamed and scarred bile ducts. Typical signs and symptoms of primary sclerosing cholangitis include :

  • Abdominal pain
  • Itchiness
  • Yellowing of your eyes and skin (jaundice)
  • Fever
  • Fatigue
  • Weight loss

Primary sclerosing cholangitis may not cause any symptoms in the early stages of the disease. In some cases, the only indication of this disorder may be abnormal blood tests suggesting that your liver isn't functioning well.

Diagnosing primary sclerosing cholangitis begins with a talk with your doctor about your signs and symptoms and your medical history. While performing a physical exam, your doctor may examine you for signs of primary sclerosing cholangitis, including an enlarged liver (hepatomegaly) and an enlarged spleen (splenomegaly). You'll likely have blood tests called liver function tests. These tests measure levels of liver enzymes, which serve as markers of your liver's health. If you have primary sclerosing cholangitis, certain enzyme levels will be abnormally high.

Other tests used to diagnose primary sclerosing cholangitis include :

  • Endoscopic retrograde cholangiopancreatography (ERCP). During this procedure, your doctor guides a flexible tube with a camera on the end (endoscope) through your esophagus and stomach to the area where the bile ducts empty into the top portion of your small intestine (duodenum). Your doctor then passes a tube down through the endoscope and injects a radioactive dye to make the bile ducts visible on X-ray pictures. If you have primary sclerosing cholangitis, your doctor will usually see narrowed bile ducts on the X-ray. ERCP is considered to be the gold standard for diagnosing primary sclerosing cholangitis.
  • Magnetic resonance cholangiography (MRC). This test uses a magnetic field and pulses of radio wave energy to make pictures of your liver and its bile ducts. This type of MRI can be done without the use of radioactive dye (contrast) and isn't as invasive as an ERCP.
  • Ultrasound. An ultrasound test uses a device that bounces sound waves off organs and structures in your body to create pictures. Changes in the bile ducts and other signs of primary sclerosing cholangitis, such as an enlarged spleen, may be visualized with this imaging test. An ultrasound is less invasive than an ERCP, but it isn't as sensitive or accurate.
  • Liver biopsy. This procedure involves obtaining a small piece of tissue for examination under a microscope. After injecting a local anesthetic, your doctor makes a small incision on your right side near your rib cage and inserts a biopsy needle to retrieve a small piece of liver tissue. By examining a liver biopsy, your doctor will be able to determine the extent of inflammation and scarring in your liver.

Because many people with primary sclerosing cholangitis also have inflammatory bowel disease, you may undergo a colonoscopy to check for inflammatory bowel disease. A colonoscopy is a procedure in which your doctor weaves a flexible tube with a camera at the end through your large intestine to check for any abnormalities. During a colonoscopy, your doctor may biopsy a piece of tissue or remove any unusual growths.

Complications :
Complications resulting from primary sclerosing cholangitis are common and may include :

  • Liver disease and failure. Chronic inflammation of the bile ducts throughout your liver can lead to tissue scarring (cirrhosis), liver cell death and, eventually, the inability of your liver to function properly.
  • Portal hypertension. One complication of liver disease is portal hypertension. This refers to high blood pressure in the portal vein, which routes blood from the lower digestive system into your liver. Portal hypertension can cause protein-containing fluid from the liver to leak into your abdominal cavity (ascites). It can also cause blood to divert from the portal vein to other veins, causing these veins to become swollen (varices). Varices are weak veins and tend to bleed easily, which can be life-threatening.
  • Bile duct cancer. Cholangiocarcinoma refers to cancer that originates in the bile ducts of your liver. Cholangiocarcinoma occurs in 10 percent to 15 percent of adults with primary sclerosing cholangitis.
  • Colorectal cancer. People with primary sclerosing cholangitis who also develop inflammatory bowel disease are at an increased risk of developing colon or rectal cancer compared with people who have either of these conditions alone.

Primary sclerosing cholangitis can't be cured with medicine or surgery. However, a number of therapies can help treat your signs and symptoms.

Medication options include :

  • Ursodiol. Ursodiol (Actigall, URSO 250) is a drug that improves symptoms in some people with primary sclerosing cholangitis; however, the long-term effects of ursodiol on the course of the disease aren't known.
  • Antibiotics. Infections within the bile ducts of the liver are common in people with primary sclerosing cholangitis. Doctors use antibiotics such as ciprofloxacin (Cipro, Cipro XR) to treat these infections.
  • Antihistamines. If you have itchiness, your doctor may recommend antihistamines (Allegra, Benadryl, others) or other medications.
  • Bile acid resins. The most common of these is cholestyramine (Questran, Questran Light). This drug binds to bile acids, helping to relieve your symptoms.

Treatment procedures include :

  • Balloon dilation and stent placement. These procedures can open blockages in the larger bile ducts. Balloon dilation is a procedure in which your doctor runs a slender tube with an inflatable balloon at its tip (balloon catheter) through an endoscope and into a blocked bile duct. Once the balloon catheter is in place, the balloon is inflated. Small plastic tubes called stents also may be placed in bile ducts to keep them open.
  • Biliary surgery. Blockages in bile ducts that can't be opened with a balloon catheter or stents may be removed surgically. After removing a blockage, your doctor connects the remaining portions of bile duct so that bile can still flow through the duct. This type of surgery is less commonly performed now because of the availability of liver transplant.
  • Liver transplant. The only cure for primary sclerosing cholangitis is a liver transplant, a surgery in which your diseased liver is removed and a healthy donor liver is inserted in its place. People with primary sclerosing cholangitis who develop liver failure or other major complications are the best candidates for liver transplantation.

Primary sclerosing cholangitis is a chronic disease that progresses slowly. While each person's disease course may vary, severe liver disease is the common endpoint. Factors associated with a poorer prognosis include :

  • Later age at diagnosis
  • Advanced disease stage as determined by liver biopsy
  • Weak and bleeding veins (variceal bleeding) and other complications
  • Coexisting inflammatory bowel disease

Fortunately, people who undergo a liver transplant and do well after the surgery have excellent chances for long-term survival.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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