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Diagnosis of PSC requires the exclusion of all secondary causes of sclerosing cholangitis; else, if a known aetiology can be uncovered, SSC is diagnosed. Its clinical and cholangiographic features may mimic PSC, yet its natural history may be more favorable if recognition is prompt and appropriate therapy is introduced.
Primary sclerosing cholangitis and primary biliary cholangitis are distinct entities and exhibit important differences, including the site of tissue damage within the liver, associations with IBD, which includes ulcerative colitis and Crohn's disease, response to treatment, and risks of disease progression. [25]
Recurrent pyogenic cholangitis (RPC), also known as Hong Kong disease, Oriental cholangitis, and Oriental infestational cholangitis, is a chronic infection characterized by recurrent bouts of bacterial cholangitis with primary hepatolithiasis. [1] [2] It is exclusive to people who live or have lived in southeast Asia. [3] [4] [5]
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. [1] [2] [3] It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis.
Murphy's sign has a high sensitivity and negative predictive value, although the specificity is not high. [2] However, in the elderly the sensitivity is markedly lower; a negative Murphy's sign in an elderly person is not useful for ruling out cholecystitis if other tests and the clinical history suggest the diagnosis.
Acute cholangitis carries a significant risk of death, the leading cause being irreversible shock with multiple organ failure (a possible complication of severe infections). [7] Improvements in diagnosis and treatment have led to a reduction in mortality: before 1980, the mortality rate was greater than 50%, but after 1980 it was 10–30%. [7]
The incidence of cholangiocarcinoma increases with age, and the disease is slightly more common in men than in women (possibly due to the higher rate of primary sclerosing cholangitis, a major risk factor, in men). [47] The prevalence of cholangiocarcinoma in people with primary sclerosing cholangitis may be as high as 30%, based on autopsy ...
If the above symptoms coincide with fever and chills, the diagnosis of ascending cholangitis may also be considered. More than 70% of people with gallstones are asymptomatic and are diagnosed incidentally during ultrasound. Studies have shown that 10% of those with gallstones will develop symptoms within 5 years of diagnosis, and 20% within 20 ...
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