Primary Sclerosing Cholangitis and Crohn’s Disease: What to Know

Crohn’s Disease and Primary Sclerosing Cholangitis: What to Know About This Liver Complication

Crohn’s Disease and Primary Sclerosing Cholangitis: What to Know About This Liver Complication
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Not only does Crohn’s disease affect the entire gastrointestinal (GI) tract, but it’s sometimes linked to a liver complication called primary sclerosing cholangitis (PSC).

 PSC is rare, affecting up to 32 in every 100,000 people, but it’s very serious and can lead to liver failure.

Although PSC has no cure, if you can catch it early, you can manage symptoms and further complications. It’s important to know the signs and symptoms and check in regularly with your healthcare provider.

What Is Primary Sclerosing Cholangitis?

PSC is a disease that causes liver inflammation and scarring (fibrosis) and narrowing of the bile ducts (which play an important part in fat digestion).

A chronic illness, PSC gets progressively worse over time, and can happen with or without inflammatory bowel diseases (IBDs) like Crohn’s.

About half of people with PSC don’t show any symptoms for a long time, but when symptoms start, they can include:

  • Right upper abdominal pain
  • Itchy skin
  • Fatigue
As PSC worsens, late symptoms that can appear may include:

  • Gastrointestinal bleeding
  • Jaundice (yellowed skin)
  • Unintentional weight loss
  • Swollen abdomen
  • Confusion
  • Enlarged liver and spleen
  • Fever and chills
Once your provider suspects PSC, they can do imaging or blood tests to diagnose it and rule out other conditions.

How Are Primary Sclerosing Cholangitis and Crohn’s Disease Linked?

PSC and Crohn’s both affect the GI tract. “Pathogenic mechanisms [causes] connecting the two have yet to be confirmed, but there are several proposed,” says Pratima Dibba, MD, a gastroenterologist in private practice in New York City.

“[These include] an autoimmune mechanism, bacterial translocation, ischemia (low oxygen) to bile ducts, genetic associations, and genetic mutations specifically affecting bile transport,” says Dr. Dibba. An imbalance in the gut microbiome may also play a role in this link.

PSC happens less often in Crohn’s disease than ulcerative colitis, another form of IBD, says Muyiwa Awoniyi, MD, PhD, a transplant hepatologist at Cleveland Clinic in Ohio. About 60 to 80 percent of people with PSC have IBD, but only 20 percent of those have Crohn’s.

 One review of 61 studies found that fewer than 1 percent of people with Crohn’s had PSC, and it was slightly more common in women.

Crohn’s disease increases your risk for colorectal cancer, and if you also have PSC, your risk goes up even more, says Dibba. As a result, you will need more frequent colonoscopies. Frequent cell turnover (development of new cells) in the GI tract caused by intestinal damage from Crohn’s increases the chance of mutations that can turn cancerous.

 The inflammation caused by PSC can boost this risk even more. Beyond colon cancer, it can also pave the way for bile duct cancer and gallbladder cancer.

How to Manage Crohn’s Disease and Primary Sclerosing Cholangitis

PSC can be unpredictable and difficult to treat.

 “There is no proven treatment to stop the disease progression of primary sclerosing cholangitis,” says James Cox, MD, a gastroenterologist and 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.

“Biologic control of Crohn’s colitis rarely changes PSC’s course,” says Dr. Awoniyi. Some experts have studied treating PSC with ursodeoxycholic acid (a medication normally used for gallstones), but experts disagree on recommending it for this purpose.

For other symptoms, your provider may suggest medications like cholestyramine (Questran) to lessen itching.

Providers sometimes recommend surgery to open up or rebuild damaged bile ducts.

PSC can eventually lead to end-stage liver disease, which can only be treated with a liver transplant.

Can You Prevent Primary Sclerosing Cholangitis if You Have Crohn’s Disease?

Whether or not you have Crohn’s disease, you can’t prevent PSC.

 But you can catch it early and manage damage to the liver.

“[People with Crohn’s disease] can monitor for the possibility of PSC by having liver enzymes done during routine checkups,” says Dibba.
You can also optimize liver health and prevent additional damage, says Cox, who recommends the following healthy lifestyle habits:

  • Limit alcohol intake, which can damage the liver over time, especially in excess.
  • Avoid supplements and herbal products that can cause liver damage, especially when taken in excess. The best way to do this is to clear it with your doctor before trying a new supplement or herbal product.
  • Maintain a healthy weight.
You can improve symptoms of PSC by eating more whole foods and fewer processed foods, doing your best to manage stress, exercising each day, and getting enough sleep.

The Takeaway

  • Primary sclerosing cholangitis (PSC) is a rare liver complication of Crohn’s disease that can cause symptoms like fatigue, itchiness, and right upper abdominal pain, among others.
  • Crohn’s disease and PSC have similar disease pathways (factors that lead the disease to develop), and both can increase your risk for certain cancers.
  • If you have Crohn’s disease, regularly check in with your healthcare provider, who can help you catch PSC early and slow its progression.
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.