National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Primary sclerosing cholangitis


Other Names:
Cholangitis, primary sclerosing; Sclerosing cholangitis
Categories:
Primary sclerosing cholangitis (PSC) is characterized by inflammation in the bile ducts (cholangitis) that leads to scarring (sclerosis), narrowing of the ducts, and a buildup of bile in the liver.[1] Early signs and symptoms include extreme tiredness, abdominal pain, and itchiness.[1] As the condition worsens it may cause jaundice, an enlarged spleen, and eventually liver cirrhosis and failure. Other complications may include weight loss, vitamin deficiency, and osteoporosis. Many people with PSC develop other autoimmune conditions such as inflammatory bowel disease, type 1 diabetes, celiac disease, or thyroid disease. PSC is also a risk factor for cancer of the bile ducts (cholangiocarcinoma).[1]
Last updated: 3/4/2013

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Autoimmunity
Autoimmune disease
Autoimmune disorder
[ more ] 		0002960
30%-79% of people have these symptoms
Abnormal large intestine physiology 0012700
Ascites
Accumulation of fluid in the abdomen
0001541
Cirrhosis
Scar tissue replaces healthy tissue in the liver
0001394
Dilated superficial abdominal veins 0030168
Elevated alkaline phosphatase of hepatic origin 0010638
Elevated hepatic transaminase
High liver enzymes
0002910
Fever 0001945
Hepatic fibrosis 0001395
Hepatomegaly
Enlarged liver
0002240
Hepatosplenomegaly
Enlarged liver and spleen
0001433
Palmar telangiectasia 0100869
Portal hypertension 0001409
Spider hemangioma 0012522
Splenomegaly
Increased spleen size
0001744
Ulcerative colitis 0100279
Weight loss 0001824
5%-29% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ] 		0002027
Adenocarcinoma of the large intestine 0040275
Celiac disease 0002608
Cholangiocarcinoma
Bile duct cancer
0030153
Cholelithiasis
Gallstones
0001081
Chronic hepatic failure
Chronic liver failure
0100626
Congestive heart failure
Cardiac failure
Cardiac failures
Heart failure
[ more ] 		0001635
Depressivity
Depression
0000716
Fatigue
Tired
Tiredness
[ more ] 		0012378
Generalized amyotrophy
Diffuse skeletal muscle wasting
Generalized muscle degeneration
Muscle atrophy, generalized
[ more ] 		0003700
Hepatitis
Liver inflammation
0012115
Hepatocellular carcinoma 0001402
Hypoalbuminemia
Low blood albumin
0003073
Jaundice
Yellow skin
Yellowing of the skin
[ more ] 		0000952
Low levels of vitamin A
Vitamin A deficiency
0004905
Low levels of vitamin D
Deficient in vitamin D
Vitamin D deficiency
[ more ] 		0100512
Low levels of vitamin E
Vitamin E deficiency
0100513
Low levels of vitamin K 0011892
Osteopenia 0000938
Osteoporosis 0000939
Pancreatitis
Pancreatic inflammation
0001733
Pleural effusion
Fluid around lungs
0002202
Polyclonal elevation of IgM 0003459
Prolonged prothrombin time 0008151
Pruritus
Itching
Itchy skin
Skin itching
[ more ] 		0000989
Renal insufficiency
Renal failure
Renal failure in adulthood
[ more ] 		0000083
Thyroiditis
Thyroid gland inflammation
0100646
Type I diabetes mellitus
Type 1 diabetes
Type I diabetes
[ more ] 		0100651
1%-4% of people have these symptoms
Acute hepatic failure
Acute liver failure
0006554
Encephalopathy 0001298
Neoplasm of the gallbladder 0100575
Uveitis 0000554
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Last updated: 7/1/2020

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include other hepatic diseases, such as autoimmune hepatitis and primary biliary cholangitis. Exclusion of multiple secondary causes leading to PSC-like bile duct changes, such as IgG4-associated sclerosing cholangitis, mast cell cholangiopathy, infections, biliary calculi or trauma, and other identifiable causes of insults to the biliary tree (leading to the ERCP or MRCP features seen in PSC) is mandatory to establish the diagnosis of PSC.
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Primary sclerosing cholangitis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for Primary sclerosing cholangitis by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Primary sclerosing cholangitis. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • What causes the pain in primary sclerosing cholangitis? Does increased pain indicate a worsening of the disease and thus the need to re-do tests? See answer


  1. Primary sclerosing cholangitis. Genetics Home Reference. May 2012; http://ghr.nlm.nih.gov/condition/primary-sclerosing-cholangitis. Accessed 3/4/2013.