Primary sclerosing cholangitis

sclerosing cholangitischolangitis, sclerosingprimary sclerosis cholangitisPSC
Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts which normally allow bile to drain from the gallbladder.wikipedia
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Inflammatory bowel disease

inflammatory bowel diseasesIBDindeterminate colitis
This is further suggested by the observation that approximately 75% of individuals with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis.
Associated complaints or diseases include arthritis, pyoderma gangrenosum, primary sclerosing cholangitis, and non-thyroidal illness syndrome (NTIS).

Cholangiocarcinoma

bile duct cancerbile ductbile duct tumor
PSC increases the risk of various cancers including liver cancer, gallbladder carcinoma, colorectal cancer, and cholangiocarcinoma. Elena Baltacha – British professional tennis player, diagnosed with PSC at age 19 and died five months after being diagnosed with PSC-associated liver cancer (specifically cholangiocarcinoma) at the age of 30. The differential diagnosis can include primary biliary cholangitis (formerly referred to as primary biliary cirrhosis), drug-induced cholestasis, cholangiocarcinoma, IgG4-related disease, post-liver transplantation non-anastomotic biliary strictures, and HIV-associated cholangiopathy. Chris LeDoux – professional rodeo rider and country musician with PSC who died of cholangiocarcinoma
Risk factors for cholangiocarcinoma include primary sclerosing cholangitis (an inflammatory disease of the bile ducts), ulcerative colitis, cirrhosis, hepatitis C, hepatitis B, infection with certain liver fluke, and some congenital liver malformations.

Cirrhosis

cirrhosis of the liverliver cirrhosisliver fibrosis
Eventually, it can lead to cirrhosis of the liver and liver failure. Portal hypertension, a complication of cirrhosis, which can manifest with esophageal and parastomal varices as well as hepatic encephalopathy (mental status alteration/disturbance caused by liver dysfunction and shunting of blood away from the scarred liver; such that ammonia detoxification is reduced with concomitant encephalopathy).
Primary sclerosing cholangitis. PSC is a progressive cholestatic disorder presenting with pruritus, steatorrhea, fat-soluble vitamin deficiencies, and metabolic bone disease. There is a strong association with inflammatory bowel disease (IBD), especially ulcerative colitis.

Ulcerative colitis

Colitiscolitis ulcerosainflammatory colitis
This is further suggested by the observation that approximately 75% of individuals with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis.
Primary sclerosing cholangitis, a distinct disease that causes inflammation of the bile ducts

Hepatomegaly

enlarged liverliver enlargementliver
Enlargement of the liver and spleen are seen in approximately 40% of affected individuals.
Primary sclerosing cholangitis.

Portal hypertension

high blood pressure in the portal systemincreased pressures in the portal vein circulationliver condition
Portal hypertension, a complication of cirrhosis, which can manifest with esophageal and parastomal varices as well as hepatic encephalopathy (mental status alteration/disturbance caused by liver dysfunction and shunting of blood away from the scarred liver; such that ammonia detoxification is reduced with concomitant encephalopathy).
Primary sclerosing cholangitis

Steatorrhea

steatorrhoeafatty stoolfatty feces
Malabsorption, especially of fat, and steatorrhea (fatty stool), due to an inadequate amount of bile reaching the small intestine, leading to decreased levels of the fat-soluble vitamins, A, D, E, and K.
Conditions affecting bile salts. Obstruction of the bile ducts by gallstones (choledocholithiasis), primary sclerosing cholangitis, liver damage (intrahepatic cholestasis), hypolipidemic drugs, or changes following gallbladder removal (cholecystectomy).

Autoimmunity

autoimmuneautoantigenauto-immune
Genetic susceptibility, immune system dysfunction, and abnormal composition of the gut flora may play a role.
A few autoimmune diseases that men are just as or more likely to develop as women include: ankylosing spondylitis, type 1 diabetes mellitus, granulomatosis with polyangiitis, Crohn's disease, Primary sclerosing cholangitis and psoriasis.

Gallbladder cancer

gallbladdergall bladder cancergallbladder carcinoma
PSC increases the risk of various cancers including liver cancer, gallbladder carcinoma, colorectal cancer, and cholangiocarcinoma.
Primary sclerosing cholangitis

Endoscopic retrograde cholangiopancreatography

ERCPcholangiopancreatography, endoscopic retrogradeendoscopic retrograde cholangio pancreatography (ERCP)
The disease was initially described in the mid-1800s but was not fully characterized until the 1970s with the advent of improved medical imaging techniques such as endoscopic retrograde cholangiopancreatography (ERCP).
Dilation of strictures (e.g. primary sclerosing cholangitis, anastomotic strictures after liver transplantation)

Elena Baltacha

Elena
Elena Baltacha – British professional tennis player, diagnosed with PSC at age 19 and died five months after being diagnosed with PSC-associated liver cancer (specifically cholangiocarcinoma) at the age of 30.
At the age of 19 she was diagnosed with the liver condition primary sclerosing cholangitis and in June 2010 she became patron of the Children's Liver Disease Foundation.

Walter Payton

PaytonWalter "Sweetness" Payton
Walter Payton – died of complications of PSC.
After struggling with the rare liver disease primary sclerosing cholangitis for several months, Payton died on November 1, 1999, aged 45, from cholangiocarcinoma.

Cholestasis

cholestaticcholestatic jaundicecholestatic hepatitis
Chronic impairment of bile flow due to blockage and dysfunctional bile transport (cholestasis) causes progressive biliary fibrosis and ultimately biliary cirrhosis and liver failure. The differential diagnosis can include primary biliary cholangitis (formerly referred to as primary biliary cirrhosis), drug-induced cholestasis, cholangiocarcinoma, IgG4-related disease, post-liver transplantation non-anastomotic biliary strictures, and HIV-associated cholangiopathy.
primary sclerosing cholangitis, associated with inflammatory bowel disease

Primary biliary cholangitis

primary biliary cirrhosisbiliary cirrhosischolestatic liver disease
The differential diagnosis can include primary biliary cholangitis (formerly referred to as primary biliary cirrhosis), drug-induced cholestasis, cholangiocarcinoma, IgG4-related disease, post-liver transplantation non-anastomotic biliary strictures, and HIV-associated cholangiopathy.
To diagnose PBC, it needs to be distinguished from other conditions with similar symptoms, such as autoimmune hepatitis or primary sclerosing cholangitis (PSC).

Chris Klug

Chris Klug – professional snowboarder with PSC who had liver transplant
After receiving a liver transplant in 2000 to treat primary sclerosing cholangitis, he went on to compete in the 2002 Winter Olympics in Salt Lake City, winning a bronze medal in the Parallel Giant Slalom.

Ursodeoxycholic acid

ursodiolUDCADeursil/Ursolvan
Some experts recommend a trial of ursodeoxycholic acid (UDCA), a bile acid occurring naturally in small quantities in humans, as it has been shown to lower elevated liver enzyme numbers in patients with PSC and has proven effective in other cholestatic liver diseases.
In double the recommended daily dose, ursodeoxycholic acid reduces elevated liver enzyme levels in those with primary sclerosing cholangitis, but its use was associated with an increased risk of serious adverse events (the development of cirrhosis, varices, death or liver transplantation) in patients who received ursodeoxycholic acid compared with those who received placebo.

Anti-neutrophil cytoplasmic antibody

ANCAanti-neutrophil cytoplasmic antibodiesantineutrophil cytoplasmic antibodies
For example, approximately 80% of people with PSC have perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA); however, this and other immunoglobulin findings are not specific to those with PSC and are of unclear clinical significance/consequence.
c-ANCA (atypical) is present in 80% of cystic fibrosis (with BPI as the target antigen) and also in inflammatory bowel disease, primary sclerosing cholangitis and rheumatoid arthritis (with antibodies to multiple antigenic targets).

Sclerosis (medicine)

sclerosisscleroticsclerotized
PSC is characterized by inflammation of the bile ducts (cholangitis) with consequent stricturing (i.e., narrowing) and hardening (sclerosis) of these ducts due to scar formation, be it inside and/or outside the liver.
Primary sclerosing cholangitis, a hardening of the bile duct by scarring and repeated inflammation.

Autoimmune hepatitis

autoimmune liver diseaseautoimmune active chronic hepatitisautoimmune chronic hepatitis
PSC associated with autoimmune hepatitis
Overlapping presentation with primary biliary cholangitis and primary sclerosing cholangitis has been observed.

Chris LeDoux

Western Underground
Chris LeDoux – professional rodeo rider and country musician with PSC who died of cholangiocarcinoma
In August 2000, LeDoux was diagnosed with primary sclerosing cholangitis, which required him to receive a liver transplant.

Liver cancer

livercancer of the liverliver tumour
Elena Baltacha – British professional tennis player, diagnosed with PSC at age 19 and died five months after being diagnosed with PSC-associated liver cancer (specifically cholangiocarcinoma) at the age of 30.
There is around 5-10% lifetime risk of cholangiocarcinoma in people with primary sclerosing cholangitis.

Bilirubin

hyperbilirubinemiaconjugated bilirubinunconjugated bilirubin
Dark urine due to excess conjugated bilirubin, which is water-soluble and excreted by the kidneys (i.e. choluria)
Primary sclerosing cholangitis

Ascending cholangitis

cholangitisacute cholangitisbiliary sepsis
Multiple episodes of life-threatening acute cholangitis (infection within the bile ducts) can be seen due to impaired drainage of the bile ducts, which increases the risk of infection.
Primary sclerosing cholangitis (an autoimmune disease leading to narrowing of the bile ducts)