PSC Of The Liver

Primary sclerosing cholangitis is inflammation of bile ducts, tubes which transport bile (digestive juice) from liver to small intestine. Chronic inflammation results in scarring of the bile ducts. Because of the scars, the ducts get narrowed and hardened.

Gradual progression of the disease is associated with higher risk of liver failure, recurrent infections and cancer of bile duct or liver.

The only treatment available for treating the disease is liver transplantation. Other treatment options are still being explored. The supportive care for primary sclerosing cholangitis involves:

• Providing symptomatic relief
• Opening the narrowed bile ducts
• Testing liver functions

Symptoms during the early stage of primary sclerosing cholangitis are non-specific and may include:

• Extreme tiredness
• Itching

As disease progresses following signs and symptoms may be experienced:

• Pain in your upper right abdomen
• Fever accompanied by shivering or chills
• Sweating during night
• Enlarged liver
• Loss of weight
• Jaundice (yellowish discoloration of your skin and eyes)

In most cases, diagnosis is made during a scheduled blood test or an X-ray, before the symptoms appear.

When to see a doctor? 

Visit your doctor if you experience signs and symptoms of primary sclerosing disease, such as extreme tiredness or persistent itchy sensation. If you have primary sclerosing disease, you are likely to develop inflammatory bowel diseases, for instance, ulcerative colitis or Crohn's disease. If you feel tired all the time or have itchy sensation and suffer from one of the inflammatory diseases, seek medical advice.

The cause of primary sclerosing cholangitis is still not understood.

Why some people are more prone to this condition could be linked to their hereditary characteristics. The disease develops as an immune response to infections or any toxic substances.

In most cases, people with primary sclerosing cholangitis may develop inflammatory bowel diseases. However, some people with inflammatory bowel disease also can develop primary sclerosing cholangitis.

The chances of developing inflammatory bowel disease after undergoing liver transplantation are very rare. 

Making a diagnosis of primary sclerosing cholangitis is done by performing several tests.

Your primary care doctor may refer you to hepatologist or gastroenterologist if you have primary sclerosing cholangitis. 

How to prepare yourself for the visit?

Getting prepared for the visit can optimize the therapy and help make the visit more fruitful.

List out all the symptoms.

Write down your key medical information. 

Write down the names of all your medications, vitamins or supplements. 

Ask a family member to accompany you during the visit.

Make a list of the questions to ask your doctor. Some typical questions can be:

• Do my test results indicate primary sclerosing cholangitis? 
• Do I need more tests? 
• How is the disease progressing? 
• How severe is my liver damage? 
• When do you think I will need a liver transplant? 
• What are treatment options for my signs and symptoms, and side effects of each treatment? 
• Do I need to test for inflammatory bowel disease? 
• How do I know my condition is worsening? 
• Are there any restrictions that I need to follow? 
• Should I see a specialist? 

What your doctor wants to know?

A clear talk with your doctor can optimize the therapy and improve the outcomes. Prepare yourself to answer some essential questions from your doctor. Your doctor might ask you typical questions like: 

• When did your start appearing? 
• How severe are your symptoms? 
• Have your symptoms been continuous or occasional? 
• Does anything improve or aggravate your symptoms?  
• How frequent is diarrhea, if any? 
• Do you have blood in your stool? 

Diagnosis of primary sclerosing cholangitis includes:

• Liver function tests: Your blood test is checked to determine the levels of liver enzymes. 
• MRI: Magnetic resonance cholangiopancreatography is a technique where your liver and bile ducts are visualized by using magnetic resonance imaging (MRI).
• X-rays of your bile ducts: Endoscopic retrograde cholangiopancreatography (ERCP), an X-ray of bile duct, may be performed alone or in combination with MRI. It involves injecting a dye into the junction between bile ducts and small intestine, through a flexible tube that runs from your throat to your small intestine. The dye makes bile duct visible on X-ray. ERCP is opted if using MRI is not possible.
• Liver Biopsy: A sample of liver tissues is removed from your liver by inserting a needle through your skin. The sample is then checked to determine the damage of your liver. Liver biopsy is performed if other tests cannot diagnose the condition. 

Treatment for primary sclerosing cholangitis is aimed at addressing the symptoms and preventing complications. Liver damage is also assessed frequently. No medications are effective in delaying or treating the liver damage. 

• Treatment for itching: Antihistamines, such as diphenhydramine and loratadine, can be used to relieve itching. It’s not clear if antihistamine are effective. However, if itching interferes with your sleep, antihistamine can be given. Antihistamine can exacerbate liver damage and make your mouth and eyes dry. 
• Bile acid binders: These medications bind to bile acids, the cause of itching. In case these drugs become ineffective or intolerable, you may be given tuberculosis drug rifampin. Rifampin reduces itching by blocking your brain's reaction to the itch-causing chemicals. Naltrexone, an opioid antagonist, also has a similar effect.
• Treatment for infections: You are likely to have recurrent bacterial infections due to accumulation of bile in the narrowed bile duct. Repeated or continuous course of antibiotics may be needed to treat the infections. Antibiotics are be prescribed if you are about to undergo procedures, such as endoscopic procedure or abdominal surgery, that make you prone to infections.
• Nutritional supplementation: Primary sclerosing cholangitis interferes with absorption of some vitamins. Your doctor may give vitamin pills or injections whenever required. Vitamin D and calcium supplements are recommended if the disease makes your bone fragile. 
• Treatment for bile duct obstruction: The obstruction of bile ducts can be treated with balloon dilation or stent placement technique. Balloon dilation technique is used to open the bile ducts that are larger and lie outside the liver. With the help of an endoscope, balloon catheter (a tube containing balloon at its tip) is placed into the obstructed duct. The balloon is then inflated. Using an endoscope, the stent is placed in the obstructed bile duct. The stent removes the blockade and allows the passage of bile acid. 
• Liver transplant: The only way to treat primary sclerosing cholangitis is a liver transplant. Your liver is replaced by healthy liver from a donor. Liver transplantation is done if you have liver failure or severe complications of primary sclerosing cholangitis. Recurrence of primary sclerosing cholangitis after liver transplantation is rare. 

Alternative remedies do not cure primary sclerosing cholangitis but may relieve some symptoms. Fatigue is one of the most common symptoms that persists even when the treatment is running. You may follow tips to beat tiredness:

• Exercise as simple as walking for 30 mins is an effective way to beat fatigue
• Stay close to friends and family 
• Manage stress by different techniques, such as meditation and relaxation exercises 
• Try yoga

If you have primary sclerosing cholangitis, following lifestyle measures may prevent further liver damage:

• Say No to alcohol,
• Get vaccinated against hepatitis A and B,
• Maintain healthy weight,
• Take medications as directed,
• Use chemicals carefully,
• Be assured that no any herbs or supplements that you are taking cause no liver damage,
• Tell your pharmacist and doctor about you liver disease before they prescribe you any drugs.

There are several risks and complications associated with primary sclerosing cholangitis.

Risks

• Age: Primary sclerosing cholangitis can affect you at any age but you are at a greater risk if you are between 30 and 50 years.
• Sex: Males are more likely to develop primary sclerosing cholangitis 
• Inflammatory bowel disease: People with primary sclerosing cholangitis may suffer from inflammatory bowel disease.
• Geographical Location: People in United States and Northern Europe have a greater risk compared to those in Asia and Southern Europe.

Complications 

• Liver disease and failure: Chronic inflammation of bile ducts can cause scarring of your liver cells and lead to death of liver cell and impaired liver function.
• Repeated infections: Accumulation of bile due to obstruction in the bile duct may cause recurrent infections. The possibility is higher if you have undergone surgery to open the blocked bile duct. 
• Portal hypertension: Increased pressure in the portal vein, a vein that conducts blood from stomach to your liver, can cause leakage of fluid from liver into the abdominal cavity. In some cases, increased pressure may cause the blood to flow from portal vein to other veins and cause swelling of these veins (varices). Varices can be fatal as they tend to bleed easily. 
• Osteoporosis: Osteoporosis (condition in which the bone loses mass and becomes weak) is one of the complications of primary sclerosing cholangitis. You may have to go for density exam to determine if you have osteoporosis. Calcium and vitamin D supplements are recommended if you have osteoporosis. 
• Bile duct cancer: You are at increased risk of having bile duct or gall bladder cancer if you are suffering from primary sclerosing cholangitis. 
• Colon cancer: Primary sclerosing cholangitis and inflammatory bowel disease together can increase the risk of colon cancer. Your doctor may test for inflammatory bowel disease if you have primary sclerosing cholangitis.