Primary sclerosing cholangitis (PSC) is a form of cholangitis due to an autoimmune reaction. A cholangitis is an inflammation of the bile ducts of the liver. Primary sclerosing cholangitis leads to cholestasis (blockage of bile transport to the gut). Blockage of the bile duct leads to accumulation of bile, which damages the liver, leading to jaundice and eventually causes liver failure. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... Cholangitis is one of a number of problems associated with the bile duct. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... An abscess on the skin, showing the redness and swelling characteristic of inflammation. ... A bile duct is any of a number of long tube-like structures that carry bile. ... For the bird, see Liver bird. ... In medicine, cholestasis is a condition where bile cannot flow from the liver to the duodenum. ... Bile (or gall) is a bitter, yellow or green alkaline fluid secreted by hepatocytes from the liver of most vertebrates. ... Jaundice, also known as icterus (attributive adjective: icteric), is a yellowing of the skin, conjunctiva (a clear covering over the sclera, or whites of the eyes) and mucous membranes caused by hyperbilirubinemia (increased levels of bilirubin in red blooded animals). ... Liver failure is the final stage of liver disease. ...

Signs and symptoms

• Tiredness (a non-specific symptom often present in liver disease)
• Severe jaundice with intense itching
• Malabsorption (especially of fat) and steatorrhea, leading to decreased levels of the fat-soluble vitamins, A, D, E and K.
• Signs of cirrhosis
• Ascending cholangitis, or infection of the bile duct.

Jaundice, also known as icterus (attributive adjective: icteric), is a yellowing of the skin, conjunctiva (a clear covering over the sclera, or whites of the eyes) and mucous membranes caused by hyperbilirubinemia (increased levels of bilirubin in red blooded animals). ... An itch (Latin: pruritus) is an uncomfortable sensation felt on an area of skin that causes a person or animal to desire to scratch that area. ... Malabsorption is the state of impaired absorption of nutrients in the small intestine. ... For other uses, see FAT. Fats consist of a wide group of compounds that are generally soluble in organic solvents and largely insoluble in water. ... Steatorrhoea is the formation of bulky, grey or pale faeces. ... Retinol (Vitamin A) For the record label, see Vitamin Records A vitamin is an organic compound required in tiny amounts for essential metabolic reactions in a living organism. ... Vitamin A is an essential human nutrient. ... Vitamin D is a fat soluble vitamin that contributes to the maintenance of normal levels of calcium and phosphorus in the bloodstream. ... Tocopherol, or Vitamin E, is a fat-soluble vitamin in eight forms that is an important antioxidant. ... Vitamin K1 (phylloquinone). ... Cirrhosis is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrotic scar tissue as well as regenerative nodules, leading to progressive loss of liver function. ... Cholangitis redirects here. ... An infection is the detrimental colonization of a host organism by a foreign species. ...

Diagnosis

The diagnosis is by imaging of the bile duct, usually in the setting of endoscopic retrograde cholangiopancreatography (ERCP, endoscopy of the bile duct and pancreas), which shows characteristic changes ("beading") of the bile ducts. Another option is magnetic resonance cholangiopancreatography (MRCP), where magnetic resonance imaging is used to visualise the biliary tract. Duodenoscopic image of two pigment stones extracted from common bile duct after sphincterotomy Fluoroscopic image of common bile duct stone seen at the time of ERCP. The stone is impacted in the distal common bile duct. ... Endoscopic images of a duodenal ulcer A flexible endoscope. ... The pancreas is a gland organ in the digestive and endocrine systems of vertebrates[2]. It is both exocrine (secreting pancreatic juice containing digestive enzymes) and endocrine (producing several important hormones, including insulin, glucagon, and somatostatin). ... MRCP is an imaging technique which detects biliary and pancreatic ducts in a non-invasive manner, moreover, after secretin stimulation, dynamic MRCP images of the pancreatic duct can be acquired. ... “MRI” redirects here. ...

Other tests often done are a full blood count, liver enzymes, bilirubin levels (usually grossly elevated), renal function, electrolytes. Fecal fat determination is occasionally ordered when the symptoms of malabsorption are prominent. A full blood count (FBC) or complete blood count (CBC) is a test requested by a doctor or other medical professional that gives information about the cells in a patients blood. ... Liver function tests (LFTs or LFs), are groups of clinical biochemistry laboratory blood assays designed to give a doctor or other health professional information about the state of a patients liver. ... Bilirubin is a yellow breakdown product of normal heme catabolism. ... In medicine (nephrology) renal function is an indication of the state of the kidney and its role in physiology. ... An electrolyte is a substance containing free ions that behaves as an electrically conductive medium. ... In medicine, a fecal fat test is a diagnostic test for fat malabsorption conditions (also referred to as steatorrhea). ...

The differential diagnosis can include primary biliary cirrhosis, drug induced cholestasis, cholangiocarcinoma, and HIV-associated cholangiopathy. This article or section does not cite its references or sources. ... In medicine, cholestasis is a condition where bile cannot flow from the liver to the duodenum. ... Cholangiocarcinoma is a cancer of the bile ducts, which drain bile from the liver into the small intestine. ... Species Human immunodeficiency virus 1 Human immunodeficiency virus 2 Human immunodeficiency virus (HIV) is a retrovirus that causes acquired immunodeficiency syndrome (AIDS, a condition in humans in which the immune system begins to fail, leading to life-threatening opportunistic infections). ...

Etiology

The cause(s) for PSC are unknown. It is often considered to be an autoimmune disorder. PSC is associated with ulcerative colitis. It is assumed that these diseases share a common cause. Autoimmunity is the failure of an organism to recognize its own constituent parts (down to the sub-molecular levels) as self, which results in an immune response against its own cells and tissues. ...

Ulcerative colitis is a systemic disease that affects many areas of the body. PSC is often listed as a manifestation of ulcerative colitis outside the colon. PSC differs from these manifestations in that, unlike most other manifestations, PSC continues in spite of surgical removal of the colon.[1] This suggests that, while the cause of ulcerative colitis, and most of its manifestations, is in the colon, the cause of PSC is located outside the colon.

Pathophysiology

Bile ducts, both intra- and extrahepatically (inside the liver and outside), are inflamed and develop scarring, obstructing the flow of bile. As bile assists in the enteric breakdown and absorption of fat, the absence of bile leads to fat malabsorption. The bile accumulates in the duct, leading to liver cell damage and liver failure.

Epidemiology

It is more prevalent in men than in women. The disease normally starts from age 30 to 60. It can however also start with children. PSC progresses slowly, so the disease can be active for a long time before it is noticed or diagnosed. This article is about the medical term. ...

Screening

PSC is associated with cholangiocarcinoma, which are tumors involving the biliary tree. Screening for cholangiocarcinoma in patients with PSC is encouraged, but there is no general consensus on the modality and interval of choice. Cholangiocarcinoma is a cancer of the bile ducts, which drain bile from the liver into the small intestine. ...

Therapy

Standard treatment includes ursodiol, a bile acid naturally produced by the liver, which has been shown to lower elevated liver enzyme numbers in people with PSC, but has not yet been proven effective at prolonging the life of the liver. Treatment also includes medication to relieve itching (antipruritics) and bile acid sequesterants (cholestyramine), antibiotics to treat infections, and vitamin supplements, as people with PSC are often deficient in vitamin A, vitamin D, and vitamin K. Ursodiol (trade names Actigall, Ursofalk, Urso Forte) is a bile acid found in large quantities in bear bile; it also occurs naturally in human bile in smaller quantities. ... Antipruritics, also known as anti-itch drugs, are medications that inhibit the itching (Latin: pruritus) that is often associated with sunburns, allergic reactions, eczema, psoriasis, chickenpox, fungal infections, insect bites and stings like those from mosquitoes, fleas, and mites, and contact dermatitis and urticaria caused by plants such as poison... Staphylococcus aureus - Antibiotics test plate. ... Retinol (Vitamin A) For the record label, see Vitamin Records A vitamin is an organic compound required in tiny amounts for essential metabolic reactions in a living organism. ... Vitamin A is an essential human nutrient. ... Vitamin D is a fat soluble vitamin that contributes to the maintenance of normal levels of calcium and phosphorus in the bloodstream. ... Vitamin K1 (phylloquinone). ...

In some cases, ERCP, which may involve stenting of the common bile duct, may be necessary in order to open major blockages (dominant strictures).

Liver transplantation (including live transplants whereby a portion of a living donor is given to the recipient) is an option if the liver begins to fail.

See also

• Bilirubin
• Child-Pugh score
• Cholangiocarcinoma
• Liver function tests
• Primary biliary cirrhosis
• Alagille syndrome
• Progressive familial intrahepatic cholestasis
• Walter Payton (died of primary sclerosing cholangitis)

Bilirubin is a yellow breakdown product of normal heme catabolism. ... In medicine (gastroenterology), the Child-Pugh score (sometimes the Child-Turcotte-Pugh score) is used to assess the prognosis of chronic liver disease, mainly cirrhosis. ... Cholangiocarcinoma is a cancer of the bile ducts, which drain bile from the liver into the small intestine. ... Liver function tests (LFTs or LFs), which include liver enzymes, are groups of clinical biochemistry laboratory blood assays designed to give information about the state of a patients liver. ... This article or section does not cite its references or sources. ... Alagille syndrome is a genetic disorder that affects the liver, heart, and other systems of the body. ... Progressive familial intrahepatic cholestasis (PFIC) refers to a group of familial cholestatic conditions caused by defects in biliary epithelial transporters. ... Walter Jerry Payton (July 25, 1954 – November 1, 1999) was an American football player, who played for the Chicago Bears of the National Football League. ...

External links

• Additional Literature about PSC
• Link to PSC Partners, a nonprofit foundation for PSC patients and caregivers
• PSC Trust is a medical research charity
• Liver Families, a pediatric liver support group
• The Morgan Foundation for the Study of PSC