Primary sclerosing cholangitis

Title

Other Names:

Cholangitis, primary sclerosing; Sclerosing cholangitis

Categories:

Digestive Diseases

Summary Summary

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

Symptoms Symptoms

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
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
Vitamin A deficiency		0004905
Vitamin D deficiency	Deficient in vitamin D	0100512
Vitamin E deficiency		0100513
Vitamin K deficiency		0011892
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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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 4/1/2019

Do you have updated information on this disease? We want to hear from you.

Related Diseases Related Diseases

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.

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 Research

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.

Organizations Organizations

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

PSC Partners Seeking a Cure
6900 E. Belleview Avenue, Suite 202
Greenwood Village, CO 80111-1627
Telephone: 303-771-5227
E-mail: contactus@pscpartners.org
Website: http://www.pscpartners.org/

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Learn More Learn More

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

Genetics Home Reference (GHR) contains information on Primary sclerosing cholangitis. This website is maintained by the National Library of Medicine.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
The National Digestive Diseases Information Clearinghouse (NDDIC), part of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), offers information on this condition. Click on the link to view information on this topic.
The Johns Hopkins Medicine Web site has an information page on primary sclerosing cholangitis. Click on the link above to view the information page.
The National Cancer Institute provides detailed information on extrahepatic bile duct cancer. Click on the link to view the information page.

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.

GARD Answers GARD Answers

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

Have a question? Contact a GARD Information Specialist.