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Hepatocyte swelling and necrosis with macrovesicular fat, Mallory bodies, and neutrophilic infiltration
4%
4/92
Periductal portal tract fibrosis and segmental stenosis of extrahepatic and intrahepatic bile ducts
37%
34/92
Centrilobular necrosis
2%
2/92
Non-suppurative destructive cholangitis and destruction of inter-lobular bile ducts
49%
45/92
Lobular disarray, ballooning degeneration, numerous apoptotic bodies, Kupffer cell activation, and lymphocyte-predominant lobular and portal inflammation
7%
6/92
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The patient description is characteristic of primary biliary cirrhosis (PBC). Non-suppurative destructive cholangitis and progressive loss of intrahepatic bile ducts are the hallmark of PBC. PBC is a chronic, non-suppurative cholangitis mainly affecting the interlobular and septal bile ducts that results in progressive ductopenia. While the exact cause is unknown, it is thought to be autoimmune in nature. About 2% of scleroderma patients have been found to have PBC in large studies. PBC is the leading cause of ductopenia, but the phenomenon can also be seen in Hodgkin's Disease, graft-verse-host disease, sarcoid, CMV infection, and HIV. In reviewing the etiologies of cirrhosis, Heidelbaugh et al. discuss using serum antibody tests to distinguish between PBC and primary sclerosing cholangitis (PSC) in patients with chronic cholangitis. Positive anti-mitochondrial antibody is associated with PBC while high titers of anti-neutrophil cytoplasmic antibody (ANCA) is associated with PSC. Scheuer describes the pathologic features and evolution of PBC and primary sclerosing cholangitis (PSC). The two disorders share many clinical and pathologic features and central to both syndromes is a substantial loss of intrahepatic bile ducts, leading to interference with bile flow. PBC biopsy specimens additionally show an element of liver-cell destruction and associated inflammation, mainly interface hepatitis that distinguishes the disorder from PSC. Interface hepatitis refers to the death of hepatocytes at the parenchymal–connective tissue interface. Illustration A shows a diagram of the four histologic stages of PBC. Illustration B shows a diagram of the intra and extrahepatic structures affected by PSC. Incorrect Answers: Answer 1: This description is characteristic of alcohol-related hepatitis or nonalcoholic fatty liver disease. Answer 2: This description is characteristic of primary sclerosing cholangitis. Answer 3: This description is characteristic of acute acetominophen toxicity. Answer 5: This description is characteristic of chronic hepatitis B.
4.9
(7)
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