Personalized Care for Patients with Primary Sclerosing Cholangitis
University of Chicago Medicine physicians collaborate closely to form a multidisciplinary team that is highly skilled in diagnosing and treating primary sclerosing cholangitis. Your treatment plan will be developed by several specialists and designed to fit your specific needs.
What is Primary Sclerosing Cholangitis (PSC)?
Primary sclerosing cholangitis (PSC) is an uncommon disease in which an overactive immune system attacks the bile ducts. Bile is an important digestive juice that is needed to break down fats and absorb vitamins A, D, E and K. It’s made in your liver and travels through thin tubes (called ducts) into your gallbladder and small intestine. With PSC, the bile ducts become inflamed and scarred, developing narrowed areas in the process and causing bile to back up into your liver. This can damage the liver.
The exact cause of primary sclerosing cholangitis isn’t clear. PSC can start at a young age; some patients have PSC for years with no symptoms, while other patients may have symptoms that lead to a diagnosis of the disease.
Primary Sclerosing Cholangitis Treatment
Currently, there is no known treatment to cure or slow the progression of primary sclerosing cholangitis. Your healthcare provider, however, can treat the symptoms and complications associated with it, such as itching and bile duct infections. PSC progresses slowly, but after roughly 10 to 15 years, it can lead to portal hypertension (high pressure in the blood vessels that drain into the liver) and cirrhosis (severe scarring of the liver), which in turn can result in liver failure. In some patients, PSC can lead to bile duct cancers. People who develop serious complications from PSC usually consider liver transplant surgery.
Frequently Asked Questions about Primary Sclerosing Cholangitis
You may have no symptoms of PSC and first learn you have it when it’s detected in a blood test. If you do have symptoms, they may develop slowly, come and go, and may – or may not – get worse over time. Bile that backs up into your liver can cause cirrhosis, which can lead to liver failure. It can also lead to bile duct infections, and long term bile duct inflammation may lead to bile duct cancer. Common symptoms of primary sclerosing cholangitis include:
- Itchy skin
- Belly pain over the liver area
- Yellowing of the skin and eyes (jaundice)
- Chills and fever (from infection in your bile ducts)
It’s believed that an overactive immune system causes primary sclerosing cholangitis, but what triggers this over-activity still isn’t clearly known. A genetic predisposition for PSC likely exists, and exposure to certain bacteria, viruses or toxins is believed to somehow trigger the immune system, which reacts in an inappropriate way and causes injury to the bile ducts. Primary sclerosing cholangitis is often diagnosed between the ages of 30 to 40, and it’s more common in men and patients with inflammatory bowel disease.
A routine blood test showing abnormal liver enzymes can help determine if you have primary sclerosing cholangitis (an increase in these enzymes is due to bile duct injury). The diagnosis can be confirmed using one of several methods:
- Magnetic resonance cholangiopancreatography (MRCP). This test uses magnetic waves and a computer program to obtain and reconstruct images of your bile ducts. Dye may be injected into your blood at the same time to obtain additional details about the liver tissue. This is the most common test used to diagnose PSC.
- Endoscopic retrograde cholangiopancreatography (ERCP). This test involves the use of an endoscope, a long, thin tube with a tiny camera and light on one end. The scope is inserted through your mouth and down into your small intestine, which allows a catheter to be inserted through the scope into the bile ducts. Dye is injected through the catheter into your bile ducts and X-ray pictures are taken. This test allows for both the diagnosis and treatment of PSC, should narrowed bile ducts be found.
- Percutaneous transhepatic cholangiography. In this study, a catheter with a needle is inserted through the skin into your liver; dye is injected into your bile ducts to obtain X-ray pictures. Similar to the ERCP, if blocked bile ducts are found, they can be opened up.
- Liver biopsy. A small sample of liver tissue is taken and checked under a microscope. It can confirm the diagnosis of PSC and provide information on the presence and severity of scar tissue. It also allows your doctor to examine your liver for other diseases.
While there is no cure for primary sclerosing cholangitis, many of its complications can be managed.
- Medications such as bile acid sequestrants and antibiotics can help relieve the itching and infections caused by PSC.
- Vitamin supplements can help your body get the nutrients you need, including vitamins for bone health. Osteoporosis (brittle bones) is common in patients with primary sclerosing cholangitis, and your bone density needs to be monitored by your doctor.
- People with primary sclerosing cholangitis are at increased risk for cancers of the bile duct, liver, gallbladder and colon. If you have inflammatory bowel disease (IBD), you should have a colonoscopy every year to check for colon cancer. Even if you do not have IBD, a colonoscopy with biopsies should be done when you are diagnosed with PSC to evaluate for IBD.
Brad's Liver Transplant Story
Starting in 2011, Brad Goodman’s health gradually declined due to a rare liver disease called primary sclerosing cholangitis (PSC). He was added to the liver transplant waiting list, but more than a year went by without an available organ. He was running out of time. UChicago Medicine doctors offered a solution.Read Brad's incredible transplant story
Why Choose UChicago Medicine for Liver Disease Care
Our internationally renowned specialists have extensive expertise in treating common and complex liver diseases.
Coordinated, Collaborative Care
Some people with liver disease have related medical problems in other areas, such as diabetes, kidney disease or heart disease. At UChicago Medicine, all of our highly specialized physicians work under the same roof and are in continual communication with one another. Whether you need care from an endocrinologist, nephrologist, cardiologist, transplant surgeon or any other specialist, you can be assured that these specialists will collaborate on your behalf.
Personalized Approach to Care
Individually tailored treatment, personalized support and ongoing education help patients through the lengthy and often challenging journey of living with liver disease. Nurse specialists, physician assistants, social workers, registered dietitians and other members of our team have important roles supporting each patient and their family throughout treatment.
A Tradition of Excellence
Today’s UChicago Medicine physicians, researchers and members of our hepatology team build on more than eight decades of experience, discoveries and treatment innovations related to liver disease. Our team oversees many clinical trials of new therapies for liver disease.
Research & Clinical Trials
Research leads to better ways to treat, diagnose and even prevent liver disease. Members of our team are actively involved in research on two fronts: in the lab (basic research) and with patients (clinical research).
In the lab, scientists are focusing on hepatitis and other liver diseases at the cellular level. Deeper understanding about how liver disease develops and progresses can lead to more effective treatments.
Clinical research often has a more timely impact on treating liver disease. As one of the nation’s leading academic hospitals, UChicago Medicine offers many clinical trials for promising new therapies. Often, these clinical trials are open to individuals whose disease has relapsed or resisted standard treatments.
Liver Transplant Institute
The University of Chicago Medicine is one of the most experienced centers for liver transplantation. Our liver transplant program was established in 1984 — the first in the Midwest and only the fourth liver transplant program in the U.S. at that time. Over the decades, transplant surgeons here have made innovations that have revolutionized liver transplantation, particularly in the area of living-donor transplant.
Ranked Among the Nation's Best
The University of Chicago Medicine has one of the premier gastroenterology and hepatology programs in the United States. Year after year, our GI program is ranked among the nation’s best for treating digestive system disorders.