Primary Sclerosing Cholangitis

Information about Primary Sclerosing Cholangitis

**Primary sclerosing cholangitis**
*Classification & external resources*
ICD-10	K83.0
ICD-9	576.1

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.

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.

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.

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.

The differential diagnosis can include primary biliary cirrhosis, drug induced cholestasis, cholangiocarcinoma, and HIV-associated cholangiopathy.

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.

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.

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.

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.

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.

External links

• • [ e] Health science - Medicine - Digestive system - Gastroenterology (primarily , )
Esophagus	GERD - Achalasia - Boerhaave syndrome - Zenker's diverticulum - Mallory-Weiss syndrome - Barrett's esophagus - Esophageal cancer - Esophageal varices
Stomach	Gastric ulcer - Non-ulcer dyspepsia - Gastroparesis - Pyloric stenosis - Malabsorption (e.g. celiac disease, giardiasis) - Stomach cancer
Small intestine	Duodenal ulcer - Intussusception - Malabsorption (e.g. celiac, lactose intolerance, fructose malabsorption, Whipple's) - Abdominal angina
Colon	Diarrhea - Appendicitis - Bowel obstruction - Diverticulitis - Diverticulosis - IBD (Crohn's, Ulcerative colitis) - IBS - Constipation - Megacolon (Toxic megacolon) - Anal fissure - Anal fistula - Anal abscess - Rectal prolapse
Hernia	Inguinal (Indirect, Direct) - Femoral - Umbilical - Incisional - Diaphragmatic - Hiatus
Liver	Alcoholic liver disease - Cholestasis - Liver failure - Cirrhosis - Hepatitis - PBC - NASH - Fatty liver - Peliosis hepatis - Portal hypertension - Hepatorenal syndrome - Budd-Chiari - Hepatocellular carcinoma
Accessory digestive	Gallbladder/Biliary tree (Gallstones, Choledocholithiasis, Cholecystitis, Cholangitis, PSC, Biliary fistula, Ascending cholangitis) - Pancreas (Acute pancreatitis, Chronic pancreatitis, Pancreatic pseudocyst, Hereditary pancreatitis, Pancreatic cancer)
Other	Tropical sprue - Hematemesis - Melena - Gastrointestinal bleeding (Upper, Lower) - Peritonitis
See also

For other uses of "ICD", see ICD (disambiguation).

The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD
..... Click the link for more information.

List of ICD-10 codes. The version for 2007 is available online at [1]

Chapter Blocks Title
I Certain infectious and parasitic diseases
II Neoplasms
III Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
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For other uses of "ICD", see ICD (disambiguation).

The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD
..... Click the link for more information.

The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. These codes are in the public domain.

Fat

Fat may refer to:

Fat, a group of compounds that are generally soluble in organic solvents and largely insoluble in water
Adipose tissue, an anatomical term for loose connective tissue composed of adipocytes

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Steatorrhoea is the formation of bulky, grey or pale faeces. Stools may also float (due to excess gas from carbohydrate malabsorption), have an oily appearance or be foul smelling. There is increased fat excretion, which can be measured by determining the faecal fat level.
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A vitamin is a nutrient that is an organic compound required in tiny amounts for essential metabolic reactions in a living organism.^[1] The term vitamin
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Vitamin A is an essential human nutrient. It exists not as a single compound, but in several forms. In foods of animal origin, the major form of vitamin A is an alcohol (retinol), but can also exist as an aldehyde (retinal), or as an acid (retinoic acid).
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Vitamin D is a group of fat-soluble prohormones, the two major forms of which are vitamin D₂ (or ergocalciferol) and vitamin D₃ (or cholecalciferol).^[1] The term vitamin D also refers to metabolites and other analogues of these substances.
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Tocopherol, known as vitamin E, describes a series of organic compounds consisting of a methylated phenols. The various derivatives are also vitamin E. Vitamin E is a fat-soluble antioxidant.
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Vitamin K denotes a group of lipophilic, and hydrophobic, vitamins that are needed for the posttranslational modification of certain proteins, mostly required for blood coagulation. Chemically they are 2-methyl-1,4-naphthoquinone derivatives.
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MeSH D008103 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.
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Ascending cholangitis
Classification & external resources

Duodenoscopy image of pus extruding from Ampulla of Vater, indicative of cholangitis
ICD-10 K 83.9
ICD-9 576.1

Ascending cholangitis is a cholangitis caused by bacterial infection.
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An infection is the detrimental colonization of a host organism by a foreign species. In an infection, the infecting organism seeks to utilize the host's resources to multiply (usually at the expense of the host).
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Endoscopic retrograde cholangiopancreatography (ERCP) is a technique that combines the use of endoscopy and fluoroscopy to diagnose and treat certain problems of the biliary or pancreatic ductal systems.
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Endoscopy means looking inside and typically refers to looking inside the human body for medical reasons using an instrument called an endoscope. Endoscopy can also refer to using a borescope in technical situations where direct line-of-sight observation is not
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The pancreas is a gland organ in the digestive and endocrine systems of vertebrates<ref name="New Standard" />. It is both exocrine (secreting pancreatic juice containing digestive enzymes) and endocrine (producing several important hormones, including
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Magnetic resonance cholangiopancreatography (MRCP) is a medical imaging technique which uses magnetic resonance imaging to visualise the biliary and pancreatic ducts in a non-invasive manner.
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