Table of ContentsView AllTable of ContentsWho’s At-RiskSymptomsAssociated DiseasesDiagnosisTreatmentsWhen to Call a Healthcare Provider
Table of ContentsView All
View All
Table of Contents
Who’s At-Risk
Symptoms
Associated Diseases
Diagnosis
Treatments
When to Call a Healthcare Provider
Primary sclerosingcholangitis(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 anautoimmunecondition. PSC is not thought to be directly inherited, but it is thought tohave 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 andcirrhosis, which prevents the liver from performing its important functions. PSC can lead to a cancerous tumor of the bile ducts calledcholangiocarcinoma, 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.
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At-Risk Demographic
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.
People with PSC are more likely to have inflammatory bowel disease (IBD) or osteoporosis.A 2019 medial study in the journalAnnals of Gastroenterologyreports 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.
People with inflammatory bowel disease who have primary sclerosing cholangitis need colonoscopy annually due to their increased risk of colon cancer.
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.
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.
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.
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, an endoscopic 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.
If any of the following symptoms occur with PSC, call your healthcare provider:
5 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M.Primary sclerosing cholangitis.The Lancet. 2018;391(10139):2547-2559. doi. 10.1016/S0140-6736(18)30300-3. Published June, 2018.Science Direct.Primary sclerosing cholangitis and inflammatory bowel disease: Intestine-liver interrelation.Mertz A.Primary sclerosing cholangitis and inflammatory bowel disease comorbidity: an update of the evidence.Annals of Gastroenterology.doi. 10.20524/aog.2019.0344. Published online January, 2019.University of Chicago Medicine.Primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD).Khoshpouri P, Habibabadi RR, Hazhirkarzar B, et al.Imaging features of primary sclerosing cholangitis: from diagnosis to liver transplant follow-up.RadioGraphics. 2019;39(7):1938-1964. doi. 10.1148/rg.2019180213. Published October, 2019.
Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M.Primary sclerosing cholangitis.The Lancet. 2018;391(10139):2547-2559. doi. 10.1016/S0140-6736(18)30300-3. Published June, 2018.
Science Direct.Primary sclerosing cholangitis and inflammatory bowel disease: Intestine-liver interrelation.
Mertz A.Primary sclerosing cholangitis and inflammatory bowel disease comorbidity: an update of the evidence.Annals of Gastroenterology.doi. 10.20524/aog.2019.0344. Published online January, 2019.
University of Chicago Medicine.Primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD).
Khoshpouri P, Habibabadi RR, Hazhirkarzar B, et al.Imaging features of primary sclerosing cholangitis: from diagnosis to liver transplant follow-up.RadioGraphics. 2019;39(7):1938-1964. doi. 10.1148/rg.2019180213. Published October, 2019.
Johns Hopkins Medicine. “Primary Sclerosing Cholangitis.” Johns Hopkins University.
The Merck Manual. “Primary Sclerosing Cholangitis (PSC).” Merck & Co, Inc.
Van Steenbergen W, De Goede E, Emonds MP, Reinders J, Tilanus M, Fevery J. “Primary Sclerosing Cholangitis in Two Brothers: Report of a Family With Special Emphasis on Molecular HLA and MICA Genotyping.“Eur J Gastroenterol Hepatol.
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