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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 .
Bile duct obstruction, which is usually present in acute cholangitis, is generally due to gallstones. 10–30% of cases, however, are due to other causes such as benign stricturing (narrowing of the bile duct without an underlying tumor), postoperative damage or an altered structure of the bile ducts such as narrowing at the site of an anastomosis (surgical connection), various tumors (cancer ...
[5] No effective medical treatment for primary sclerosing cholangitis is known. Its most definitive treatment is a liver transplant, [1] but disease recurrence can occur in 25-30% of cases. [6] PSC is a rare disease and most commonly affects people with IBD. [2]
The treatment of RPC involves management of sepsis during episodes of cholangitis with antibiotics, abscess drainage, and blood pressure support. With resistant infection, a surgical hepatectomy or hepaticocutaneousjejunostomy can be performed. Lifelong surveillance for malignancy is also usually necessary. [15]
SSC is a sclerosing cholangitis with a known cause. Alternatively, if no cause can be identified, then primary sclerosing cholangitis is diagnosed. SSC is an aggressive and rare disease with complex and multiple causes. It is characterized by inflammation, fibrosis, destruction of the biliary tree and biliary cirrhosis.
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Without treatment mortality rates of autoimmune enteropathy are as high as 30%. [8] Many factors such as the need for parenteral nutrition, [61] age of presentation, and the severity of symptoms can impact long-term outcomes. [3] No one treatment has been proven successful in all cases and relapses are common. [4]
AGS can cause a range of symptoms: mild ones like a rash or hives, or more severe ones such as difficulty breathing, and even anaphylaxis, says infectious disease expert Amesh A. Adalja, M.D ...