Sclerosing cholangitis is a chronic liver disease characterized by inflammation and scarring of the bile ducts. These ducts are the pipelines that carry bile, a digestive fluid produced by the liver, from the liver to the gallbladder and the small intestine.

In a healthy person, these ducts are smooth, open tubes. In sclerosing cholangitis, a chronic inflammatory process attacks the lining of the bile ducts. The body’s attempt to heal this constant inflammation leads to the formation of dense scar tissue (fibrosis). This scarring causes the ducts to narrow in multiple places, creating strictures. This process obstructs the normal flow of bile, a condition known as cholestasis.

A helpful analogy is to think of the bile ducts as a complex system of flexible drainage pipes. In sclerosing cholangitis, it’s as if a corrosive substance is constantly flowing through the hoses. This causes the inside of the hoses to become inflamed and scarred. Over time, the flexible hose walls become thick, hard, and rigid, and multiple kinks and narrow spots develop. This prevents fluid from draining properly, causing it to back up into the liver. This backup of toxic bile damages the liver cells, leading to further scarring of the liver itself (fibrosis), and can eventually progress to cirrhosis.

There are two main types of sclerosing cholangitis:

• Primary Sclerosing Cholangitis (PSC): This is the most common form. It is a chronic disease where the cause of the bile duct inflammation is unknown, though it is believed to be autoimmune in nature. It is strongly associated with inflammatory bowel disease.
• Secondary Sclerosing Cholangitis (SSC): In this type, the bile duct damage is a result of a known, identifiable cause, such as a previous injury, infection, or blockage.

When I explain this condition to patients, I emphasize that the inflammation is silent at first but the long-term effects on the liver can be serious without regular monitoring and care.

The specific triggers and mechanisms for this disease differ between the primary and secondary forms.

Causes of Primary Sclerosing Cholangitis (PSC)

The exact cause of PSC is unknown. It is thought to be a complex, multifactorial autoimmune disease.

Causes of Secondary Sclerosing Cholangitis (SSC)

In SSC, the damage to the bile ducts is a direct result of a known, preceding insult. The causes can include:

• Ischemic Injury: A lack of blood flow to the bile ducts, which can be a complication of liver transplantation or other major abdominal surgeries.
• Recurrent Bacterial Cholangitis: Repeated, severe bacterial infections of the bile ducts, often caused by long-standing blockages from gallstones or previous surgical strictures.
• Direct Trauma: Injury to the bile ducts during a surgical procedure, such as gallbladder removal.
• Certain Infections in people with weakened immune systems.

In practice, I’ve seen patients diagnosed after years of managing ulcerative colitis. It’s often discovered during routine liver tests, even before symptoms appear.

Because the causes are different, the risk factors for PSC and SSC are also different. This article will primarily focus on the more common PSC.

Primary Sclerosing Cholangitis is not contagious. You cannot catch it from another person. Disease development is linked to a specific set of risk factors.

• Having Inflammatory Bowel Disease (IBD): This is the single strongest risk factor. Approximately 75% of all people diagnosed with PSC also have a form of IBD, most commonly ulcerative colitis. The link between the gut inflammation of IBD and the bile duct inflammation of PSC is an area of intense research.
• Age: PSC is most often diagnosed in people aged 30 to 40.
• Gender: The disease is more common in men than women, with men being affected about twice as often.
• Genetics: PSC has a clear genetic component. While it doesn’t follow a simple inheritance pattern, having a first-degree relative with the condition increases a person’s risk.

I often tell patients with PSC that while we don’t fully understand the trigger, it tends to follow a pattern especially in those with inflammatory bowel disease. SSC, on the other hand, usually has a clear external cause.

Early symptoms of PSC are often mild or absent. The disease is frequently discovered incidentally on routine blood tests that show abnormal liver function, often performed as part of the monitoring for a patient’s known inflammatory bowel disease.

When symptoms do develop, the most common early signs are:

• Fatigue: A profound and persistent sense of tiredness.
• Pruritus: Severe, widespread skin itching that can be debilitating.
• Upper Abdominal Pain: A dull, aching pain in the upper right quadrant of the abdomen.

As the bile ducts become more scarred and blocked and liver damage progresses, other symptoms will appear.

• Jaundice (yellowing of the skin and eyes) and dark urine.
• Fever, chills, and shaking (signs of an acute bacterial cholangitis, a serious complication).

In the advanced stages, as the liver becomes severely scarred (cirrhotic), symptoms of liver failure may develop, such as fluid buildup in the abdomen (ascites), swelling in the legs (edema), and mental confusion (hepatic encephalopathy).

What I’ve often seen is that fatigue or itching are the earliest signs, and they’re easy to overlook. By the time jaundice appears, significant liver damage may already be present.

A doctor, usually a gastroenterologist or hepatologist, will suspect PSC in a patient, especially a man with ulcerative colitis, who has unexplained abnormal liver tests.

• Initial Blood Tests: The first clue is a liver function test (LFT) that shows a characteristic cholestatic pattern. This means the level of the enzyme Alkaline Phosphatase (ALP) is significantly elevated.
• Cholangiography (Imaging the Bile Ducts): The definitive diagnosis of PSC is made by visualizing the bile ducts with imaging.
  • Magnetic Resonance Cholangiopancreatography (MRCP): This is the preferred, first-line, and non-invasive test for diagnosing PSC. It is a specialized type of MRI scan that creates highly detailed images of the biliary tree. In PSC, the MRCP will show the classic “beads on a string” appearance of multiple strictures and dilations throughout the intrahepatic and extrahepatic bile ducts.
  • Endoscopic Retrograde Cholangiopancreatography (ERCP): This is an invasive procedure where an endoscope is passed through the mouth to the small intestine, and dye is injected directly into the bile ducts for an X-ray. While it provides excellent images, ERCP carries a risk of complications like pancreatitis. Today, it is used more for therapy than initial diagnosis.
• Liver Biopsy: A biopsy is not always needed to diagnose PSC, but it may be performed to assess the stage of liver damage and fibrosis.
• Colonoscopy: Because of the extremely strong association, it is recommended that every person newly diagnosed with PSC undergo a colonoscopy to screen for underlying inflammatory bowel disease.

The classic “beading” appearance of bile ducts on MRCP is almost diagnostic. I always follow up with blood work and monitor progression closely because it can change quickly, even when symptoms are stable.

There is no cure for PSC. Treatment focuses on managing symptoms, slowing disease progression, and preventing complications.

1. Medical Therapy

• Ursodeoxycholic Acid (UDCA): This is an oral medication, a type of bile acid, that is widely prescribed for PSC. It can improve liver blood test results, but its ability to improve long-term survival or slow the progression to cirrhosis is debated and has not been conclusively proven in large clinical trials (American College of Gastroenterology, 2022).
• Symptom Management: Medications are used to treat the debilitating itching. Prompt treatment with antibiotics is essential for any episode of bacterial cholangitis.

2. Endoscopic Therapy

For patients who develop a single, severe, or “dominant” stricture in a large bile duct, an ERCP can be used as a therapeutic tool. During the procedure, a doctor can use a balloon to dilate the narrowed area and may place a small plastic or metal tube called a stent to hold the duct open and improve bile drainage.

3. Surveillance for Complications

This is a critical part of long-term management.

• Cancer Surveillance: People with PSC have a significantly increased lifetime risk of developing cholangiocarcinoma (cancer of the bile ducts), and gallbladder cancer. Regular surveillance with imaging (such as ultrasound or MRI) and blood tests is performed to try to detect these cancers early.
• Colon Cancer Surveillance: Due to the high prevalence of ulcerative colitis in PSC patients, they also have a very high risk of developing colon cancer. Regular screening colonoscopies at frequent intervals are mandatory.
• Bone Density Screening: Patients are at risk for osteoporosis and should be monitored with bone density scans.

4. Liver Transplantation

For patients whose disease progresses to end-stage liver failure (cirrhosis with severe complications), a liver transplant is the only definitive, life-saving treatment. Transplant outcomes for PSC are generally very good. However, there is a risk that the disease can recur in the transplanted liver over time.

Managing PSC is about playing the long game. I reassure patients that even without a cure, we can often keep symptoms controlled for years and transplant is a highly successful option when needed.

Primary Sclerosing Cholangitis is a rare, chronic, and serious liver disease that results from the progressive inflammatory destruction of the bile ducts. It is strongly linked with inflammatory bowel disease and carries a significant long-term risk of severe complications, including liver failure and cancer. While the diagnosis of a progressive disease with no cure can be daunting, the focus of modern medicine is on a proactive, lifelong strategy of surveillance and symptom management. I always tell patients, sclerosing cholangitis may be slow-moving, but with careful monitoring and modern treatments, it doesn’t have to control your life and when needed, liver transplant offers a fresh start.

American Liver Foundation. (n.d.). Primary Sclerosing Cholangitis (PSC). Retrieved from https://liverfoundation.org/liver-diseases/primary-sclerosing-cholangitis-psc/

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). (2022). Primary Sclerosing Cholangitis. Retrieved from https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis

American College of Gastroenterology (ACG). (2022). Primary Sclerosing Cholangitis. Retrieved from https://gi.org/topics/primary-sclerosing-cholangitis/