IBD and Primary Sclerosing Cholangitis (PSC)

Primary sclerosing cholangitis (PSC) is a rare and chronic disease of the liver that causes inflammation and narrowing of the bile ducts inside and outside of the liver. It's not certain what causes PSC, although it is thought to be an autoimmune condition. PSC is not thought to be directly inherited, but it is thought to have a genetic component.

Bile is necessary for the digestion of fat and to carry waste out of the liver. PSC causes the bile ducts to narrow from scarring and inflammation, and bile begins to accumulate in the liver, which damages it. This damage eventually leads to scar formation and cirrhosis, which prevents the liver from performing its important functions. PSC can lead to a cancerous tumor of the bile ducts called cholangiocarcinoma, which occurs in 10 to 15% of patients.

PSC does progress slowly in most cases, but it can also be unpredictable and life-threatening. People with PSC can receive treatment to relieve symptoms and help them lead an active life. 

At-Risk Demographic

PSC is most often diagnosed by age 40 and is affects men twice as much as women for reasons unknown. On the whole, PSC is an uncommon disease.

Symptoms

Some people have no symptoms at diagnosis or even for several years after, according to the Mayo Clinic, reporting early signs of PSC include fatigue, itching, jaundice, and abdominal pain. The symptoms worsen as the disorder progresses with the following signs becoming more common.

• Fever
• Chills
• Night sweats
• Enlarged liver
• Enlarged spleen
• Weight loss

Associated Diseases

People with PSC are more likely to have inflammatory bowel disease (IBD) or osteoporosis. A 2019 medial study in the journal Annals of Gastroenterology reports IBD and PSC "are closely associated disease entities that, when present in combination, create a phenotypically different summative disease referred to as PSC-IBD." According to the study, approximately 70% of patients with PSC suffer from underlying IBD, specifically ulcerative colitis.

Diagnosis

There are a number of ways to diagnose PSC, according to John Hopkins Medicine, reporting laboratory tests, a liver biopsy, endoscopic retrograde cholangiopancreatography (ERCP), and magnetic resonance cholangiopancreatography (magnetic resonance cholangiography (MRCP).

During an ERCP, the healthcare provider inserts an endoscope into the mouth and navigates it down through the esophagus and stomach to the ducts of the biliary tree. Dye is inserted into the ducts so that they will show up when x-rays are taken. The x-rays are then analyzed to determine if there are any problems with the bile ducts.

Less intrusive, laboratory tests can also detect PSC by abnormal serum levels in the blood. A liver biopsy can be useful when the imaging modalities are not diagnostic or when overlap syndrome is suspected. This procedure is done in a hospital setting on an outpatient basis with a local anesthetic. The healthcare provider performing the test will use a needle to take a small sample of liver tissue for testing by a pathologist.

Lastly, a MRCP, specialized magnetic resonance imaging, can be used to detect the rare condition. A 2019 medical study explains the noninvasive imaging does not require x-rays like an ERCP, so there is no radiation exposure to the patient. "MRCP has high diagnostic sensitivity (86%) and specificity (94%) for detection of PSC," according to the study.

Treatments

There is no definitive treatment for PSC. Research to find an effective medical treatment is currently underway. In the meanwhile, treatment plans focus on alleviating symptoms, interrupting the progression of the disease, and monitoring for potential complications.

The Mayo Clinic reports types of treatment for the itching and infection caused by PSC. For the symptom of itching, bile acid sequestrants, antibiotics, antihistamines, opioid antagonists, and ursodeoxycholic acid are used.

For recurrent infections that may occur with PSC, antibiotics could be necessary. The Mayo Clinic reports that PSC hinders vitamin absorption, and supplementing fat-soluble vitamins, including A, D, E, and K, can aid in deficiency.

Lastly, the Mayo Clinic reports If blockages occur in the bile ducts, a surgical procedure may be necessary to stretch or open them. Stents, which keep the ducts open, may be placed in the ducts during this procedure or balloon dilation may be used. If the progression of the PSC leads to liver failure or persistent biliary infections, a liver transplant may be necessary. Liver transplants offer a good quality of life for recipients.

When to Call a Healthcare Provider

If any of the following symptoms occur with PSC, call your healthcare provider:

• Abdominal pain
• Black or very dark stools
• Jaundice
• Temperature over 100.4
• Vomiting with blood