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Alcohol-induced liver disease
Alpha 1-antitrypsin deficiency
Benign liver tumors
Cholestatic liver disease
Non-alcoholic fatty liver disease
Primary biliary cirrhosis
Primary sclerosing cholangitis
Pancreatic neuroendocrine neoplasms
Familial adenomatous polyposis
Juvenile polyposis syndrome
Small bowel ischemia and infarction
Protein losing enteropathy
Short bowel syndrome (NORD)
Small bowel bacterial overgrowth syndrome
Diverticulosis and diverticulitis
Irritable bowel syndrome
Cleft lip and palate
Congenital diaphragmatic hernia
Diffuse esophageal spasm
Eosinophilic esophagitis (NORD)
Gastroesophageal reflux disease (GERD)
Cyclic vomiting syndrome
Gastric dumping syndrome
Dental caries disease
Gingivitis and periodontitis
Temporomandibular joint dysfunction
Appendicitis: Pathology review
Cirrhosis: Pathology review
Colorectal polyps and cancer: Pathology review
Congenital gastrointestinal disorders: Pathology review
Diverticular disease: Pathology review
Esophageal disorders: Pathology review
Gallbladder disorders: Pathology review
Gastrointestinal bleeding: Pathology review
GERD, peptic ulcers, gastritis, and stomach cancer: Pathology review
Inflammatory bowel disease: Pathology review
Jaundice: Pathology review
Malabsorption syndromes: Pathology review
Neuroendocrine tumors of the gastrointestinal system: Pathology review
Pancreatitis: Pathology review
Viral hepatitis: Pathology review
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Primary Sclerosing Cholangitis - A Patient's Story
Primary Sclerosing Cholangitis Labs and Treatment
Primary Sclerosing Cholangitis Mechanisms
Autoimmune Biliary Tract Disorders & Cancer
sclerosing cholangitis p. 402
sclerosing cholangitis and p. 402
in sclerosing cholangitis p. 402
ulcerative colitis p. 389
ulcerative colitis association p. 389
The name “primary sclerosing cholangitis” or PSC, is actually pretty straightforward, primary refers to it not being known to have been caused by anything else, in other words it isn’t secondary to something else.
Sclerosing means hardening of the tissue and cholangitis is inflammation of the bile ducts.
So with PSC we have this fibrosis and inflammation of both the intra- and extra-hepatic ducts, so inside and outside the liver.
As these cells around the bile ducts become inflamed, die, and harden due to fibrosis, you get this like tightening of the ducts in some areas where there’s been serious fibrosis, and then dilation in other areas that aren’t as affected, and this leads to the classic PSC finding of this sort of “beaded” appearance of the bile ducts.
These areas of fibrosis in the bile ducts can also be seen on histology, and looks a bit like an onion skin since you’ve got these concentric rings of fibrosis around the bile duct, so sometimes it’s referred to as “onion-skin fibrosis”.
Again, with PSC, we don’t really know what causes it right? One clue though is that it’s been known to be associated with ulcerative colitis which is an autoimmune disease and crohn’s disease which is immune-system related; knowing that, PSC’s thought to possibly be an autoimmune disorder itself involving the immune system’s T cells attacking and destroying bile duct epithelial cells.
Why they might start doing this, though, is not very well known, and likely happens in people with certain genetic predispositions when they’re exposed to some specific stimuli in their environment.
Certain genetic factors have been linked to developing PSC; studies have shown that patients with PSC tend to have in common specific human leukocyte antigens, or HLAs.
HLAs are these specific markers on cells that tell your body whether these cells are your own cells or someone else’s cells, and sometimes, for reasons that are usually not well known, the body’s immune system might think that certain HLAs or cell surface markers are foreign even when they’re your own, leading to an autoimmune disorder.
Specific HLA’s that have been common among people with PSC are HLA-B8, HLA-DR3, and HLA-DRw52a.
Another piece of supporting evidence for the autoimmune theory is that most patients with PSC have elevated IgM antibody levels in the serum, as well as an antibody called p-ANCA.
Primary sclerosing cholangitis (PSC) is a disease of the bile ducts that causes inflammation and obliterative fibrosis of bile ducts inside and/or outside of the liver. This results in scarring of the biliary tree, which impedes the flow of bile to the intestines and can ultimately lead to cirrhosis of the liver, liver failure, and liver cancer. Symptoms of PSC can include fatigue, itching, jaundice, abdominal pain, and weight loss. If left untreated, PSC can lead to liver failure. Definitive treatment is liver transplant, but ursodeoxycholic acid (UDCA help to slow disease progression and alleviate symptoms.
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