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Primary sclerosing cholangitis (PSC) is a chronic liver disease caused by progressive inflammation and scarring of the bile ducts of the liver. The inflammation impedes the flow of bile to the gut, which can ultimately lead to liver cirrhosis and liver failure. The underlying cause of the inflammation is believed to be autoimmunity. [1] The definitive treatment is liver transplantation.
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.
Most patients with PSC have evidence of autoantibodies. Approximately 80% of patients have perinuclear antineutrophil cytoplasmic antibodies, also called p-ANCA, however this finding is not specific for PSC. Antinuclear antibodies and anti-smooth muscle antibodies are found in 20%-50% of PSC patients and, likewise, are not specific for the disease. [1]
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.
The cause(s) for PSC are unknown. It is often considered to be an autoimmune disorder. PSC is associated with inflammatory bowel disease and particularly ulcerative colitis, which coexists in approximately 70% of patients. Conversely, the prevalence of PSC in ulcerative colitis patients is ~4%. There is a 2:1 male-to-female predilection of PSC. [2] There is an increased prevalence of HLA alleles A1, B8, and DR3 in PSC. [1]
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