Primary Sclerosing Cholangitis and Ulcerative Colitis: What to Know

Primary Sclerosing Cholangitis and Ulcerative Colitis: What to Know About This Liver Complication

Primary Sclerosing Cholangitis and Ulcerative Colitis: What to Know About This Liver Complication
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When you have ulcerative colitis (UC), a chronic inflammatory disease that affects the large intestine, you can be at risk for a rare liver complication called primary sclerosing cholangitis (PSC). As PSC progresses, it can cause liver inflammation, scarring, and bile duct problems and may eventually lead to liver failure.

Although PSC has no cure, you can manage liver damage with early diagnosis and treatment. Here’s how PSC and UC are connected, and what you can do if you have both.

What Is Primary Sclerosing Cholangitis?

Like UC, PSC is a chronic inflammatory condition that occurs when the immune system attacks and damages the body. This rare condition only occurs in up to 32 out of every 100,000 people.

 Damage from PSC can happen in the liver and bile ducts — pathways used to carry bile to the intestines where they break down fats.

Inflammation in these ducts can eventually lead to scarring, which makes the ducts narrow and obstructs the flow of bile. Over time, this can damage the liver more and more until the organ fails.

Almost 50 percent of people with PSC have no symptoms at the time of diagnosis, but if they do, they can include:

  • Right upper abdominal pain
  • Itchy skin
  • Fatigue
As PSC progresses, more severe symptoms can appear, including:

  • Gastrointestinal bleeding
  • Jaundice (yellowed skin)
  • Unintentional weight loss
  • Swollen abdomen
  • Confusion
  • Enlarged liver and spleen
  • Fever and chills
If your provider thinks you could have PSC, they may order imaging or blood tests to confirm or rule out the diagnosis.

How Are Primary Sclerosing Cholangitis and Ulcerative Colitis Linked?

Experts don’t fully understand what causes PSC, but they think inflammatory bowel diseases (IBDs) like UC are a major risk factor in its development. “Primary sclerosing cholangitis (PSC) and ulcerative colitis (UC) are tightly intertwined,” says Muyiwa Awoniyi, MD, PhD, a transplant hepatologist at Cleveland Clinic in Ohio.

Up to 80 percent of people diagnosed with PSC also have an IBD, and of those, 80 percent have UC.

 “The mechanistic link appears to lie along the gut-liver axis,” says Dr. Awoniyi. Imbalances in the gut microbiome, increased intestinal permeability (the ability of the intestinal lining to control the passage of substances from the gut into the bloodstream), and bacteria relocation (movement of bacteria from one place to another) may trigger bile duct inflammation, he adds.

In people with UC and PSC, symptoms like inflammation in the colon are more likely to spare the rectum, says Awoniyi. “[But they carry] a markedly elevated colorectal cancer risk.” When you have UC, your bowel lining must make new cells more frequently to respond to damage, which allows more chances for cancer-causing mutations to develop.

 When you add in PSC inflammation, the risk goes up even more, and people with the condition need more frequent colonoscopies.

People with PSC and UC also have a higher risk of developing bile duct cancer and gallbladder cancer than if they had UC alone.

How to Manage Ulcerative Colitis and Primary Sclerosing Cholangitis

“The treatment of ulcerative colitis is not affected by the presence of primary sclerosing cholangitis,” says James Cox, MD, a gastroenterologist, an assistant professor, and the director of clinical skills development and physician communication at Burnett School of Medicine at Texas Christian University in Fort Worth. But PSC treatments can be offered alongside UC treatment.

Treatments can lessen symptoms and complications of PSC, but no options yet exist to slow it down or cure it.

 Some experts recommend ursodeoxycholic acid (UDCA) for PSC, a medication normally used to treat gallstones, but the research on its effectiveness for PSC is limited.

Also, UC treatments don’t reliably treat the liver, says Awoniyi. Standard UC immunosuppressants and biologics can spark healing in the intestinal lining, but rarely change PSC progression, he adds. But researchers are testing alternative approaches. “Several small studies of oral antibiotics — most notably vancomycin — show meaningful reductions in colitis activity (clinical and endoscopic) in PSC‑UC,” says Awoniyi.

Depending on your symptoms, your provider may recommend a therapeutic endoscopy.

 When PSC narrows your bile ducts, endoscopic treatment is used to widen your ducts with a balloon, which can not only prevent damage to the bile ducts but also offer an opportunity to take a biopsy and rule out cancer, says Dr. Cox.
PSC progresses slowly, but often ends in severe liver disease that may require liver transplantation within 15 to 20 years after diagnosis.

 In fact, PSC is the fifth most common reason for liver transplant in the United States.

 “Thus, management centers on aggressive colitis surveillance (annual colonoscopy after diagnosis), individualized use of antibiotics, and vigilant hepatobiliary monitoring rather than expecting liver benefit from conventional UC strategies,” says Awoniyi.

Can You Prevent Primary Sclerosing Cholangitis if You Have Ulcerative Colitis?

PSC is not preventable, but taking good care of your liver can slow additional damage and even improve symptoms like fatigue. To protect your liver, follow these healthy lifestyle habits:

“The best we can offer is meticulous control of intestinal inflammation … [and] steps that may mitigate downstream complications even if they do not demonstrably avert PSC,” says Awoniyi, who encourages taking your medication, optimizing nutrition, and considering participation in microbiome- or bile acid–targeted trials for future treatment options.

The Takeaway

  • Primary sclerosing cholangitis (PSC), a rare liver complication of ulcerative colitis (UC), is a chronic inflammatory disease that progressively damages the liver and bile ducts over time.
  • Early symptoms of PSC with UC include fatigue, itchiness, and abdominal pain. More severe symptoms include jaundice, intestinal bleeding, and confusion, among others.
  • PSC can’t be prevented or cured, but early diagnosis and treatment can lessen symptoms and delay further complications.
EDITORIAL SOURCES
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Resources
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Yuying Luo, MD

Medical Reviewer

Yuying Luo, MD, is an assistant professor of medicine at Mount Sinai West and Morningside in New York City. She aims to deliver evidence-based, patient-centered, and holistic care for her patients.

Her clinical and research focus includes patients with disorders of gut-brain interaction such as irritable bowel syndrome and functional dyspepsia; patients with lower gastrointestinal motility (constipation) disorders and defecatory and anorectal disorders (such as dyssynergic defecation); and women’s gastrointestinal health.

She graduated from Harvard with a bachelor's degree in molecular and cellular biology and received her MD from the NYU Grossman School of Medicine. She completed her residency in internal medicine at the Icahn School of Medicine at Mount Sinai, where she was also chief resident. She completed her gastroenterology fellowship at Mount Sinai Hospital and was also chief fellow.

Abby McCoy, RN

Author

Abby McCoy is an experienced registered nurse who has worked with adults and pediatric patients encompassing trauma, orthopedics, home care, transplant, and case management. She is a married mother of four and loves the circus — that is her home! She has family all over the world, and loves to travel as much as possible.

McCoy has written for publications like Remedy Health Media, Sleepopolis, and Expectful. She is passionate about health education and loves using her experience and knowledge in her writing.