What Are Primary Sclerosing Cholangitis (PSC) and Primary Biliary Cirrhosis (PBC)?

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rimary sclerosing cholangitis is a chronic disorder. It affects the liver with inflammation that leads to permanent scarring (cirrhosis) and inhibits liver function. How the disorder develops is unclear, but it may arise after unrelated infections. Scarring is its principal feature.

Primary biliary cirrhosis is a chronic autoimmune disease. It produces progressive destruction of the liver’s bile ducts by the immune system. This begins with inflammation, which can progress and reduce liver function. Medication delays the disorder’s advance.

Both diseases have similar symptoms and prognosis. However, they develop for different reasons, produce damage in different ways, and are prevalent in distinct patient populations.

What Conditions Are Primary Sclerosing Cholangitis and Primary Biliary Cirrhosis Associated With?

Primary sclerosing cholangitis produces fever, chills, and abdominal pain. Inability of the body to properly manage bile may turn the skin yellow temporarily. The majority of people with the disorder also have inflammatory bowel disease. Experts believe there is a strong genetic aspect to who develops the condition, but this disposition does not guarantee someone will get it.

Primary biliary cirrhosis has symptoms similar to primary sclerosing cholangitis. Symptoms may develop slowly over five to 20 years. In the most severe cases, a liver transplant is required. Diagnosis is most likely after age 40. Approximately 90% of all patients with the disorder are women, and prevalence is higher in those of European descent.

Many cases of both primary biliary cirrhosis and primary sclerosing cholangitis are diagnosed before symptoms are severe. This is because about 70% of primary biliary cirrhosis patients experience serious fatigue that leads them to see a doctor. Fatigue is also the most common symptom in its sister disease and tends to be noticed first.

Neither condition should be confused with acute cholangitis. Acute cholangitis is characterized by bile duct damage caused by temporary blockage of the ducts. The blockage inhibits bile flow, which leads to infection. Acute cholangitis requires emergency hospitalization.

Primary Sclerosing Cholangitis and Primary Biliary Cirrhosis Lifestyle Changes and Recovery

Primary sclerosing cholangitis and primary biliary cirrhosis are forms of liver disease. Lifestyle changes may be necessary based on the liver damage. Patients with either disease often benefit from discontinuing alcohol use, removing a possible source of liver damage.

No cure exists for these diseases. Care focuses on monitoring and managing complications over time. With appropriate medical intervention, liver function can be preserved for many years. The appropriate treatments differ based on the patient’s age and comorbid conditions.

What Research Currently Exists Around Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis?

Ongoing medical research about primary biliary cirrhosis focuses on understanding the genetic underpinnings of the disorder: Its incidence, prevalence, and changes in blood test results that can predict its presence during the asymptomatic stages.

Research about primary sclerosing cholangitis is launched regularly. In 2016, the disease saw its largest-ever genetic study. That work produced new findings pertaining to four parts of the genome associated with the disease, potentially pointing the way to treatment.

Current Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis Clinical Trials

The following primary biliary cirrhosis clinical studies and primary sclerosing cholangitis clinical trials come from ClinicalTrials.gov. If you have a clinical study you would like featured, please contact our team.

Conclusion

PBC and PSC are rare and relatively mysterious. Clinical trials are essential for developing primary biliary cirrhosis clinical resources that could arrest progress of the disease. Likewise, new medical research is the clearest path to a cure for primary sclerosing cholangitis.