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Primary Sclerosing Cholangitis - Causes, Symptoms, Diagnosis, and Treatment

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Primary sclerosing cholangitis is a disorder obstructing bile in intrahepatic and extrahepatic bile ducts. The article explains the disorder.

Medically reviewed by

Dr. Jagdish Singh

Published At October 12, 2022
Reviewed AtMay 15, 2023

Introduction:

Primary sclerosing cholangitis is a complex disorder that was first mentioned and described in medical literature in 1867. Primary sclerosing cholangitis is a rapidly evolving disease that may be associated with other conditions. The characteristic features of the disease are the abnormal formation of fibrous tissue in the bile ducts, inflammation, and enlargement of the bile ducts.

Both the intrahepatic and extrahepatic bile ducts are affected by primary sclerosing cholangitis. Symptoms of primary sclerosing cholangitis include itching, fatigue, and jaundice (yellowing mucous membrane discoloration, skin, and skin whites of the eyes). Affected persons may also suffer from nausea or abdominal pain and pass dark urine and light-colored stools. The treatment for primary sclerosing cholangitis is not specified, however, treatment of appearing symptoms helps to slow the progression of the disease.

What Are the Causes of Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis is a multifactorial disorder that is caused by a combination of several factors such as environmental, genetic, and immunologic.

  • Researchers state that primary sclerosing cholangitis results from a triggering factor that is not specific and causes an abnormal immune response that leads to an autoimmune reaction. The body’s immune system attacks its bile duct cells, leading to primary sclerosing cholangitis. In most cases, the triggering factors are toxic agents or any infection.

  • Genetics has an important role in the onset of primary sclerosing cholangitis as the incidence of the disease is very high among first-degree relatives such as parents, children, and siblings. Researchers have stated that there are 16 genetic regions that can be expected to be associated with primary sclerosing cholangitis. Genetic predisposition means that a person carries genes for the disease which gets triggered due to an external environmental factor.

  • Several theories explain the pathogenesis of primary sclerosing cholangitis including leaky gut syndrome in individuals with primary sclerosing cholangitis along with inflammatory bowel disease or the toxic bile theory. But these theories can not explain the cause of all the cases of primary sclerosing cholangitis. Thus, more research and studies must be carried out to know the exact cause of primary sclerosing cholangitis.

Which Population Is Mostly Affected by Primary Sclerosing Cholangitis?

Though primary sclerosing cholangitis is a rare disease, it affects males twice as often as females. The age spectrum is not specific as it can affect any age group. Gradually primary sclerosing cholangitis is becoming one of the main reasons behind liver transplant cases in the United States of America.

What Are the Symptoms of Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis leads to episodes of interrupted or obstructed bile flow from the liver, known as cholestasis. This obstruction occurs due to thickening, inflammation, and formation of abnormal fibrous tissues within the bile ducts. Initially, the patient may be asymptomatic or show only mild symptoms which progress gradually.

Some of the common symptoms of primary sclerosing cholangitis are:

  • Abdominal discomfort and pain, especially in the upper right side of the abdomen.

  • Fatigue or tiredness.

  • Pruritus: Uncomfortable and irritating sensation, creating an urge to scratch the skin.

  • Obstructive Jaundice: Blockage of bile flow from the liver leads to absorption of bile into the bloodstream. This results in yellowishness of the skin, mucous membrane, and sclera of the eyes.

  • Passing of dark-colored urine and light-colored stools.

  • Nausea: Feeling of uneasiness and vomiting.

  • Hepatomegaly: Abnormal enlargement of the liver.

  • Splenomegaly: Abnormal enlargement of the spleen.

  • Sudden weight loss.

  • Deficiency of fat-soluble vitamins including A, D, E, and K.

  • Hepatic Osteodystrophy: A metabolic bone disease that develops due to chronic liver disease.

  • The affected patients may also suffer from osteoporosis, in which the bone density and calcium deposition in bones are reduced. Osteoporosis leads to repeated fractures.

  • Episodes of fever, chills, and night sweating due to bacterial cholangitis.

  • Scarring and destruction of liver tissues due to bile accumulation.

  • Portal Hypertension: Increased blood pressure in the vessels of the liver.

  • Ascites: Ascites is defined as the accumulation of fluid in the abdomen.

  • Increased chances of development of cholangiocarcinoma. Approximately 8-15 % of affected individuals with primary sclerosing cholangitis develop cholangiocarcinoma.

  • Ulcerative colitis and inflammatory bowel disease in almost 60% to 70% of cases of primary sclerosing cholangitis.

Some of the Autoimmune Diseases Associated With Primary Sclerosing Cholangitis Are:

  • Celiac Disease: It is also known as gluten-sensitive enteropathy. It is an immune response to eating gluten.

  • Sjorgen’s Syndrome: Immune disorder is characterized by the common symptoms of dry mouth and eyes.

  • Peyronie’s Disease: Non-cancerous condition resulting from fibrous scar tissue developed on the penis and causes painful and curved erections.

  • Psoriasis: A skin disease that commonly causes itchy, scaly, and red patches on elbows, knees, scalp, and trunk. It is a chronic disease with no cure.

  • Wegener’s Granulomatosis: It is an uncommon disorder that leads to inflammation of the blood vessels in which the throat, sinuses, lungs, kidneys, and nose.

  • Pyoderma Gangrenosum: It is a rare condition characterized by large, painful ulcers that develop on the skin and legs.

How to Diagnose Primary Sclerosing Cholangitis?

The most important step in diagnosing primary sclerosing cholangitis is a medical history and clinical examination. After this, there are a few tests that help to confirm the diagnosis.

These are:

  • Liver Functional Test: It is performed to evaluate the concentration and levels of liver enzymes. Elevation of liver enzymes such as alkaline phosphatase, aspartate aminotransferase, alanine aminotransferase, and gamma-glutamyl transpeptidase indicate chances of liver disease but specifically primary sclerosing cholangitis.

  • Magnetic Resonance Cholangiopancreatography: It is a non-invasive test used to evaluate the extrahepatic and intrahepatic bile ducts.

  • Endoscopic Retrograde Cholangiopancreatography: This imaging technique also helps to diagnose primary sclerosing cholangitis. An endoscope (a thin flexible tube) is inserted into the mouth and down through the esophagus, stomach, and bile ducts. A contrast dye is taken to investigate and evaluate the function and health of bile ducts.

  • Liver Biopsy: A sample of tissue is taken surgically for a microscopic examination to evaluate the progress of primary sclerosing cholangitis and the state of the liver.

  • In some cases, a colonoscopy is recommended to evaluate the function and health of the bowels due to the association of primary sclerosing cholangitis with inflammatory bowel disease and colon cancer.

What Is the Treatment for Primary Sclerosing Cholangitis?

There is no specific treatment for primary sclerosing cholangitis, however, treating the associated symptoms can slow the progress of the disease.

  • Narrowed or blocked bile ducts can be treated with medical procedures, such as percutaneous transhepatic cholangiography and endoscopic retrograde cholangiopancreatography.

  • Itchy skin is cured by skin creams and lotions containing Menthol, camphor, Pramoxine, or Capsaicin. In some cases, antihistamines such as Fexofenadine can also be used.

  • Supplements for fat-soluble vitamin deficiency and calcium deficiency in bones.

  • Antibiotics such as Penicillin are advised for bile duct infections.

  • In cirrhosis or liver failure cases, transplantation is the only treatment choice.

Conclusion:

Primary sclerosing cholangitis is a complex disorder with no specific causative factor. It affects both intrahepatic and extrahepatic bile ducts. The episodic obstruction of bile due to the disease leads to symptoms such as inflammation, abdominal pain, scarring, and cirrhosis of the liver. The treatment for primary sclerosing cholangitis depends on the treatment of associated symptoms.

Frequently Asked Questions

1.

What Is the Average Life Expectancy for Individuals with Primary Sclerosing Cholangitis?

 
The average life expectancy for individuals with primary sclerosing cholangitis varies widely. It is influenced by factors such as the stage of the disease, the presence of complications, and the response to treatment. Studies have shown a reduced life expectancy compared to the general population, but with advancements in medical management and liver transplantation, some patients can live for many years. Regular monitoring, adherence to treatment plans, and early intervention are important.

2.

What Are the Recommended Treatment Options for Primary Sclerosing Cholangitis?

 
The recommended treatment options for primary sclerosing cholangitis (PSC) depend on the individual's symptoms, disease severity, and complications. There is no known cure for PSC, but treatment aims to manage symptoms, slow disease progression, and prevent complications. Medications such as Ursodeoxycholic acid may be prescribed to alleviate symptoms and improve liver function. In advanced cases, liver transplantation is often considered the most effective treatment option. Additionally, managing associated conditions, such as inflammatory bowel disease, and maintaining a healthy lifestyle are important components of PSC treatment.

3.

Is It Possible to Recover from Primary Sclerosing Cholangitis?

 
Recovery from primary sclerosing cholangitis (PSC) is rare, as it is a chronic and progressive condition. Treatment focuses on managing symptoms, slowing disease progression, and preventing complications. Adherence to treatment plans and lifestyle modifications can improve quality of life and delay disease progression.

4.

At What Age Is Primary Sclerosing Cholangitis Typically Diagnosed?

Primary sclerosing cholangitis (PSC) is typically diagnosed in adulthood, usually between the ages of 30 and 50. However, it can occur at any age. 

5.

Can Individuals with Primary Sclerosing Cholangitis Lead a Normal Life?

Individuals with primary sclerosing cholangitis (PSC) can live relatively normally, but the disease may be challenging. The prognosis and impact on daily life can vary depending on symptoms, disease progression, and complications. With proper management, individuals can maintain an active and fulfilling life.

6.

Is It Possible to Live with Primary Biliary Cholangitis for 20 Years?

Primary biliary cholangitis (PBC) is a distinct liver disease from primary sclerosing cholangitis (PSC). PBC is a chronic autoimmune condition that primarily affects the small bile ducts in the liver. If left untreated, PBC can progress over time, potentially leading to liver cirrhosis or liver failure. However, with appropriate management, including medication such as Ursodeoxycholic acid, lifestyle modifications, and regular monitoring, many individuals with PBC can live with the condition for 20 years or more.

7.

What Foods Should Be Avoided in Individuals with PSC?

 
In individuals with primary sclerosing cholangitis (PSC), avoiding certain foods that may aggravate symptoms or put additional strain on the liver is generally recommended. A balanced and nutritious diet with plenty of fruits, vegetables, lean proteins, and whole grains is typically encouraged. Working with a registered dietitian can help individuals with PSC develop a personalized dietary plan that meets their needs and supports overall liver health.

8.

Which Blood Tests Are Used to Diagnose PSC?

Several blood tests diagnose primary sclerosing cholangitis (PSC) and assess liver function. These tests include liver function tests (LFTs), which measure various enzymes and proteins in the blood to evaluate liver health. Additionally, blood tests may be conducted to check for specific autoantibodies associated with PSC, such as antinuclear antibodies (ANA) and anti-smooth muscle antibodies (ASMA). Elevated levels of alkaline phosphatase (ALP) and bilirubin, along with other abnormalities in LFTs, can provide important diagnostic information.

9.

Does a Liver Transplant Provide a Cure for Primary Sclerosing Cholangitis?

 
Liver transplantation is the only known cure for primary sclerosing cholangitis (PSC). A liver transplant provides a chance for a significantly improved quality of life and long-term survival for individuals with advanced PSC and liver failure. However, it is important to note that some cases of PSC can recur in the transplanted liver. Careful evaluation and selection of suitable candidates, close post-transplant monitoring, and adherence to immunosuppressive medications are crucial for successful outcomes. The decision to pursue a liver transplant should be made in consultation with a transplant specialist based on individual circumstances and disease progression.

10.

What Are the Risks Associated with Primary Sclerosing Cholangitis?

 
Primary sclerosing cholangitis (PSC) carries several risks and complications. The disease is linked with an increased risk of developing other conditions, such as inflammatory bowel disease (IBD) and hepatobiliary malignancies, including cholangiocarcinoma. PSC can also lead to progressive liver damage, resulting in cirrhosis, liver failure, and the need for liver transplantation. In some cases, PSC can cause complications such as bile duct strictures, recurrent cholangitis (bile duct infection), and portal hypertension. Regular monitoring, early detection of complications, and appropriate medical interventions are important for managing these risks and optimizing outcomes for individuals with PSC.

11.

Can a Liver Biopsy Be Used to Detect PSC?

 
Sometimes, a liver biopsy can be used as a diagnostic tool to detect primary sclerosing cholangitis (PSC). A small sample of liver tissue is obtained and examined under a microscope to assess the presence of inflammation, fibrosis, and characteristic changes associated with PSC during a liver biopsy. However, a liver biopsy is not always necessary for diagnosing PSC. Other diagnostic methods, such as imaging tests like magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP), blood tests, and clinical evaluation, can often provide sufficient information for a diagnosis. The decision to perform a liver biopsy is typically based on the individual's specific clinical presentation and the need for further disease severity confirmation or assessment.

12.

What Is the Survival Rate for Individuals with Primary Sclerosing Cholangitis?

 
The survival rate for individuals with primary sclerosing cholangitis (PSC) can vary depending on various factors, such as the stage of the disease, the presence of complications, and the individual's response to treatment. Studies have shown that PSC can have a variable and unpredictable disease course, with some individuals experiencing relatively stable conditions for many years while others may progress more rapidly. The 10-year survival rate for PSC ranges from around 60 percent to 80 percent, but it is important to note that individual outcomes can differ significantly. Regular medical monitoring, adherence to treatment plans, and timely interventions are crucial for improving long-term survival rates in individuals with PSC.

13.

Is Primary Sclerosing Cholangitis a Life-Threatening Condition?

 
Primary sclerosing cholangitis (PSC) can be life-threatening, particularly in advanced stages or when complications arise. PSC is a chronic and progressive disease that leads to liver cirrhosis, liver failure, and an increased risk of developing hepatobiliary malignancies. The severity and prognosis of PSC can vary among individuals. Early diagnosis, close monitoring, and appropriate medical management are crucial for minimizing the risk of life-threatening complications and improving long-term outcomes for individuals with PSC. Individuals with PSC need to work closely with their healthcare team to optimize their treatment and overall well-being.

14.

At What Age Does Primary Sclerosing Cholangitis Typically Occur in the Liver?

 
Primary sclerosing cholangitis (PSC) can occur at any age, but it is most commonly diagnosed in young to middle-aged adults, typically between the ages of 30 and 50. However, it is important to note that PSC can also be diagnosed in children and older individuals. The exact age of onset can vary among individuals, and diagnosis is based on a combination of clinical evaluation, symptoms, imaging tests, and laboratory findings. If PSC is suspected, further evaluation by a healthcare professional is necessary to confirm the diagnosis and initiate appropriate management.

15.

Can a Computed Tomography Scan Be Used to Detect Primary Sclerosing Cholangitis?

A CT (computed tomography) scan can help detect primary sclerosing cholangitis (PSC) by providing detailed images of the liver and bile ducts. While a CT scan is not the primary diagnostic tool for PSC, it can be useful in evaluating the liver and identifying potential signs of PSC, such as bile duct dilation, liver fibrosis, or cirrhosis. However, imaging tests like magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP) are more commonly used to visualize the bile ducts and confirm a diagnosis of PSC. A healthcare professional will find the most appropriate imaging modality based on individual circumstances and clinical presentation.

16.

How Rapidly Does Primary Sclerosing Cholangitis Progress?

 
Primary sclerosing cholangitis (PSC) progression can vary widely among individuals. In some cases, the disease progresses slowly over many years, while in others, it may progress more rapidly. Various factors, including the severity of inflammation, the presence of complications, and the response to treatment, can influence the rate of progression. Regular monitoring, close medical supervision, and adherence to treatment plans are essential for assessing disease progression and intervening promptly when necessary. However, it is important to note that the specific rate of progression and its predictability can vary from person to person.

17.

Which Medications Are Commonly Prescribed for PSC in the Liver?

 
Treating primary sclerosing cholangitis (PSC) in the liver typically involves a combination of medications and supportive care. Ursodeoxycholic acid (UDCA) is commonly prescribed to manage symptoms and slow disease progression. It may help improve liver function and reduce inflammation in some cases. Other medications, such as immunosuppressants or anti-inflammatory drugs, may be used to manage specific symptoms or address complications associated with PSC. Additionally, lifestyle modifications, including a healthy diet, regular exercise, and avoiding alcohol, are important for overall liver health. The medication regimen will depend on individual circumstances and should be determined by a healthcare professional.
Dr. Jagdish Singh
Dr. Jagdish Singh

Medical Gastroenterology

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bile duct diseaseprimary sclerosing cholangitis
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