Description

Primary sclerosing (skluh-ROHS-ing cholangitis (koh-lan-JIE-tis) is a disease of the bile ducts. The bile ducts carry the digestive fluid bile from the liver to the small intestine. In the primary sclerosing cholangitis, the inflammation causes scarring within the bile ducts. These scars make the ducts, hard and narrow and gradually cause serious damage to the liver. The majority of people with primary sclerosing cholangitis also have inflammatory bowel disease such as ulcerative colitis or Crohn's disease.

In the majority of people with primary sclerosing cholangitis, the disease progresses slowly. Eventually, it can lead to liver failure, repeated infections, and tumors of the biliary tract or the liver. A liver transplant is the only known cure for advanced primary sclerosing cholangitis, but the disease can recur in the transplanted liver in a small number of patients.

Care of the primary sclerosing cholangitis is focused on the monitoring of the liver function, the management of symptoms and, when possible, to perform procedures that temporarily open obstruction of the bile ducts.

Symptoms

Primary sclerosing cholangitis is often diagnosed before symptoms appear when a routine blood test or an x-ray taken by a relationship of condition shown abnormalities in the liver.

The first signs and symptoms often include:

  • Fatigue
  • Itching
  • Yellow eyes and skin (jaundice)
  • Abdominal Pain

Many people with a diagnosis of primary sclerosing cholangitis before they have symptoms continue to feel well, in general for several years. But there is no reliable way to predict how quickly or slowly the disease progress to any person.

The signs and symptoms that may appear as the disease progresses include:

  • Fever
  • Chills
  • Night sweats
  • Enlargement of the liver
  • Enlargement of the spleen
  • Weight loss

When to see a doctor

Make an appointment with your doctor if you have severe, unexplained itching in the greater part of his body — itching that persists no matter how much you scrape. Also consult with your doctor if you feel very tired all the time, no matter what you do.

It is particularly important to carry unexplained fatigue and itching to the attention of your doctor if you have ulcerative colitis or Crohn's disease, both of which are types of inflammatory bowel disease. The majority of people with primary sclerosing cholangitis also have one of these diseases.

Causes

It is not clear what the cause of primary sclerosing cholangitis. An immune system reaction to an infection or a toxin can trigger the disease in people who are genetically predisposed to it.

A large proportion of people with primary sclerosing cholangitis also have inflammatory bowel disease, an umbrella term that includes ulcerative colitis and Crohn's disease.

Primary sclerosing cholangitis and inflammatory bowel disease does not always appear at the same time, however. In some cases, the primary sclerosing cholangitis is present for years before the inflammatory bowel disease occurs. If primary sclerosing cholangitis is diagnosed, it is important to seek the inflammatory bowel disease because there is a greater risk of colon cancer.

A little less often, people who are receiving treatment for inflammatory bowel disease have primary sclerosing cholangitis as well. And rarely, individuals with primary sclerosing cholangitis develop inflammatory bowel disease only after a liver transplant.

Risk factors

Factors that may increase the risk of primary sclerosing cholangitis include:

  • Age. Primary sclerosing cholangitis can occur at any age, but is most often diagnosed between the ages of 30 and 40 years.
  • Sex. Primary sclerosing cholangitis occurs more frequently in men.
  • The inflammatory bowel disease. A large proportion of people with primary sclerosing cholangitis also have inflammatory bowel disease.
  • The geographic location. People with Northern European heritage have an increased risk of primary sclerosing cholangitis.

Complications

Complications of primary sclerosing cholangitis may include:

  • Liver disease and failure. The chronic inflammation of the bile ducts along their liver can lead to tissue scarring (cirrhosis), liver cell death and, finally, the loss of function of the liver.
  • The repeated infections. If the scarring of the bile ducts that decreases or stops the flow of bile from the liver, you may experience frequent infections in the bile ducts. The risk of infection is especially high after having had a surgical procedure to expand a badly scarred bile duct or to remove a stone blockage of a bile duct.
  • The Portal hypertension.The portal vein is the main pathway for the blood that flows to your digestive system, your liver. Portal hypertension refers to high blood pressure in this vein. Portal hypertension can cause fluid from the liver to leak into your abdominal cavity (ascites). You can also divert the blood from the portal vein to other veins, making these veins are swollen (varicose veins). Varicose veins are weak veins and tend to bleed easily, which can be life-threatening.
  • Thinning of the bones. People with primary sclerosing cholangitis may experience thinning of the bones (osteoporosis). Your doctor may recommend a bone density test to test for osteoporosis every few years. Calcium and vitamin D supplements may be prescribed to help prevent bone loss.
  • Cancer of the bile duct. If you have primary sclerosing cholangitis, have a higher risk of developing cancer in the bile ducts or gallbladder.
  • The Colon cancer. People with primary sclerosing cholangitis associated with inflammatory bowel disease have an increased risk of colon cancer. If you have been diagnosed with primary sclerosing cholangitis, the doctor may recommend testing for inflammatory bowel disease, even if you have no signs or symptoms, since the risk of colon cancer is high if you have both diseases.

The Portal hypertension. The portal vein is the main pathway for the blood that flows to your digestive system, your liver. Portal hypertension refers to high blood pressure in this vein.

Portal hypertension can cause fluid from the liver to leak into your abdominal cavity (ascites). You can also divert the blood from the portal vein to other veins, making these veins are swollen (varicose veins). Varicose veins are weak veins and tend to bleed easily, which can be life-threatening.

Diagnosis

Tests and procedures used to diagnose primary sclerosing cholangitis include:

  • The liver function blood test. A blood test to check liver function, including the levels of liver enzymes, you can give your doctor clues about your diagnosis.
  • MRIof the bile ducts. Magnetic resonance cholangiopancreatography (koh-lan-jee-o-pan-cree-uh-TOG-ruh-fee) using magnetic resonance imaging (MRI) to take pictures of your liver and the bile ducts and is the test of choice to diagnose primary sclerosing cholangitis.
  • X-rays of your bile ducts.A type of bile duct X-ray called an endoscopic retrograde cholangiopancreatography (ERCP), in addition to, or instead of, anMRImay be necessary. But this test is rarely used for the diagnosis, due to the risk of complications. To make the bile ducts visible on x-ray, the doctor uses a flexible tube that is passed through the throat to inject a contrast dye into the area of your small intestine, where the bile ducts empty. AnERCPis the test of choice if signs and symptoms persist despite the fact that there are no anomalies in anMRI. AnERCPis often the initial test, if you are unable to have anMRIbecause of a metal implant in your body.
  • The liver biopsy.A liver biopsy is a procedure to remove a piece of liver tissue for laboratory testing. The doctor inserts a needle through the skin and into the liver to remove a sample of tissue. A liver biopsy can help determine the extent of damage to your liver. The test is used only when the diagnosis of primary sclerosing cholangitis is still uncertain after the less invasive tests.

X-rays of your bile ducts. A type of bile duct X-ray called an endoscopic retrograde cholangiopancreatography (ERCP), in addition to, or instead of, an mri may be needed. But this test is rarely used for the diagnosis, due to the risk of complications.

To make the bile ducts visible on x-ray, the doctor uses a flexible tube that is passed through the throat to inject a contrast dye into the area of your small intestine, where the bile ducts empty.

ERCP is the test of choice if signs and symptoms persist despite the fact that there are no abnormalities on an mri . An ERCP is often the initial test, if you can't do an mri due to a metal implant in your body.

The liver biopsy. A liver biopsy is a procedure to remove a piece of liver tissue for laboratory testing. The doctor inserts a needle through the skin and into the liver to remove a sample of tissue.

A liver biopsy can help determine the extent of damage to your liver. The test is used only when the diagnosis of primary sclerosing cholangitis is still uncertain after the less invasive tests.

Treatment

Treatments for primary sclerosing cholangitis focus on the management of complications and the monitoring of damage in the liver. Many medicines have not been studied in patients with primary sclerosing cholangitis, but until now have not been found to reduce or reverse the liver damage associated with this disease.

The treatment for itching

  • Bile acid sequestrants. Drugs that bind to bile acids — substances that are believed to cause itching in liver disease — are the first line of treatment for itching in the primary sclerosing cholangitis.
  • Antibiotics. If you have difficulty tolerating a bile acid binding of the drug, or if it does not help, your doctor may prescribe rifampin (Rifadin, Rimactane, other), an antibacterial drug. Exactly how to rifampicin reduces the itching is unknown, but it can block the brain's response to the itch-inducing chemicals in their circulation.
  • Antihistamines.This type of medication can help reduce the itching caused by primary sclerosing cholangitis. If these drugs are effective for this disease is unknown. Antihistamines can worsen liver disease symptoms of dry eyes and dry mouth. On the other hand, antihistamines may help with sleep, if the itching is kept awake.
  • Opioid antagonists. The itching associated with liver disease may also respond to an opioid antagonist drugs, such as naltrexone. As rifampicin, these drugs seem to reduce the itching sensation of acting on your brain.
  • Ursodeoxycholic acid (UDCA). Also known as ursodiol, UDCA is a natural form of bile acids, which may help relieve the itching symptoms caused by a disease of the liver by increasing the absorption capacity of the bile.

Antihistamines. This type of medication can help reduce the itching caused by primary sclerosing cholangitis. If these drugs are effective for this disease is unknown.

Antihistamines can worsen liver disease symptoms of dry eyes and dry mouth. On the other hand, antihistamines may help with sleep, if the itching is kept awake.

The treatment for infections

The bile that accumulates in the narrowing or blockage of the ducts of the common causes of bacterial infections. To prevent and treat these infections, people with primary sclerosing cholangitis may take repeated courses of antibiotics, or to continue taking antibiotics for long periods.

Before any procedure that could cause an infection, such as an endoscopic procedure or abdominal surgery, you will also need to take antibiotics.

Nutritional support

Primary sclerosing cholangitis makes it difficult for your body to absorb certain vitamins. Although you can eat a healthy diet, you may find that you can't get all the nutrients you need.

Your doctor may recommend vitamin supplements that you take in tablet form, or that you receive as an infusion through a vein in your arm. If the disease that weakens the bones, you can take calcium and vitamin D supplements as well.

The treatment of bile duct obstructions

The crashes that occur in the bile ducts may be due to progression of the disease, but it can be a sign of cancer of the biliary tract. Endoscopic retrograde cholangiopancreatography (ERCP) can help you to determine the cause, and the obstruction of bile ducts can be treated with:

  • The balloon dilation. This procedure can open up blockages in the larger bile ducts outside the liver. In the balloon dilation, your doctor will run a thin tube with an inflatable balloon at its end (the balloon of the catheter) through an endoscope and a blockage of the bile ducts. Once the balloon of the catheter is in place, the balloon is inflated.
  • The placement of a Stent. In this procedure, the doctor uses an endoscope and is attached instruments to place a small plastic tube, called a stent in an obstruction of the bile duct to keep the duct open.

Liver transplant

A liver transplant is the only treatment known to cure of primary sclerosing cholangitis. During a liver transplant, surgeons remove the diseased liver and replace it with a healthy liver from a donor.

A liver transplant is reserved for people with liver failure or other serious complications of primary sclerosing cholangitis. Although rare, it is possible that the primary sclerosing cholangitis to reappear after a liver transplant.

Lifestyle and home remedies

If you have been diagnosed with primary sclerosing cholangitis, take steps to take care of the liver, such as:

  • Do not drink alcohol.
  • Get vaccinated against hepatitis a and B.
  • Careful with the chemicals in the home and in the workplace.
  • Maintain a healthy weight.
  • Follow the instructions of all medications, both prescription and over-the-counter. Make sure that your pharmacist and any doctor who prescribes it for you to know that you have a disease of the liver.
  • Talk with your doctor about any herbs or supplements you are taking, as some can be harmful to the liver.

Alternative medicine

There is No alternative medicine treatments have been found for the treatment of primary sclerosing cholangitis. But some complementary and alternative therapies may help you cope with signs and symptoms of the disease. Talk with your doctor about your options.

Fatigue is common in people with primary sclerosing cholangitis. While doctors can treat some of the factors that can contribute to fatigue, their signs and symptoms can persist. You can find relief with complementary and alternative treatments that have shown some benefit to the fatigue, such as:

  • Regular exercise for more than two hours before going to bed, which can help to promote better sleep
  • A well-balanced diet that includes fruits, vegetables, whole grains and proteins
  • Stress management techniques such as meditation and relaxation exercises

Preparing for your appointment

Start by seeing your primary care doctor if you have signs or symptoms that worry you. If your doctor suspects that you may have primary sclerosing cholangitis, you may be referred to a liver specialist (gastroenterologist or hepatologist).

Because appointments can be brief, and because there is often a lot of ground to cover, it is a good idea to be well prepared. Here's some information to help you prepare and what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. When you make your appointment, be sure to ask if there is anything that you need to do in advance, such as restrict your diet.
  • Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • Write down key personal information, including any major stresses or recent life changes.
  • Make a list of all the medicines, as well as any vitamins or supplements you are taking.
  • Consider the possibility of a family member or friend. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write questions to ask their doctor.

Your time with your doctor is limited. You may feel less rushed if you make a list of questions you want to ask at your appointment. Here are some of the possible questions about the primary sclerosing cholangitis:

  • Can you explain the results of my test for me?
  • More evidence is needed?
  • Up to what point my primary sclerosing cholangitis progressed?
  • How serious is the damage to my liver?
  • You can estimate when you may need a liver transplant?
  • What treatments can relieve my symptoms and signs?
  • What are the possible side effects of each treatment?
  • Should I be tested for inflammatory bowel disease?
  • What are the signs and symptoms of the signal that my condition is getting worse and I need to make another appointment?
  • Are there any restrictions that I need to follow?
  • You should see a specialist?
  • Are there brochures or other printed material that I can take with me? What sites do you recommend?

In addition to the questions that you've prepared to ask your doctor, do not hesitate to ask questions during your appointment.

What to expect from your doctor

Your doctor may ask you a series of questions. Be ready to answer them may allow more time later to cover the points you want to address. Your doctor may ask:

  • When did you begin experiencing symptoms?
  • The symptoms been continuous or occasional?
  • How severe are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • Do you have frequent episodes of diarrhea?
  • Have you noticed blood in your stool?
Symptoms and treatment of Primary sclerosing cholangitis