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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]
Reynolds' pentad is a collection of signs and symptoms consistent with obstructive ascending cholangitis, a serious infection of the biliary tract.It is a combination of Charcot's triad (right upper quadrant pain, jaundice, and fever) with shock (low blood pressure, tachycardia) and an altered mental status. [1]
Charcot's cholangitis triad is the combination of jaundice; fever, usually with rigors; and right upper quadrant abdominal pain. It occurs as a result of ascending cholangitis (an infection of the bile duct in the liver). When the presentation also includes low blood pressure and mental status changes, it is known as Reynolds' pentad. [1]
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]
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.
Cholestatic pruritus is the sensation of itch due to nearly any liver disease, but the most commonly associated entities are primary biliary cholangitis, primary sclerosing cholangitis, obstructive choledocholithiasis, carcinoma of the bile duct, cholestasis (also see drug-induced pruritus), and chronic hepatitis C viral infection and other forms of viral hepatitis.
Anti-mitochondrial antibodies (AMA) are autoantibodies, consisting of immunoglobulins formed against mitochondria, [1] primarily the mitochondria in cells of the liver.. The presence of AMA in the blood or serum of a person may be indicative of the presence of, or the potential to develop, the autoimmune disease primary biliary cholangitis (PBC; previously known as primary biliary cirrhosis).
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]