Journal of Pathology and Translational Medicine

Original Article

Clinicopathologic Comparison between Autoimmune Cholangitis and Primary Biliary Cirrhosis.: Gyeong Hoon Kang, So Dug Lim, Eun Sil Yu, On Ja Kim, Geun Chan Lee, Neung Hwa Park, Dong Jin Suh; Korean J Pathol. 1998;32(2):115-124.

Abstract PDF: Primary biliary cirrhosis (PBC) is characterized by histological findings of an immunoinflammatory destruction of small- and medium-sized bile ducts with progressive portal fibrosis, and the presence of anti-mitochondrial antibody (AMA) with a laboratory evidence of chronic cholestasis. The term "autoimmune cholangitis" (AIC) is used for a disease with the clinical and pathologic features of primary biliary cirrhosis (PBC) but with negative AMA and positive anti-nuclear antibody (ANA) tests. Eight cases of AIC and ten cases of PBC were reviewed in order to determine whether there was any difference between two diseases in clinico-pathologic aspects. All of the patients were female and the mean ages of AIC and PBC patients were 48 and 47 years, respectively. ANA test was positive in six of ten PBC paients and their mean titer was lower than that of AIC patients. IgM level was significantly higher in PBC group than in AIC group. No significant difference was found between two groups with respect to biochemical and histopathological features. Since the only consistently distinguishing features between these two conditions are the autoantibody profile (AMA vs ANA) and immunoglobulin level (IgM), these two conditions might be part of a spectrum. PBC can be considered to be the same as AMA-positive AIC or alternatively AIC to be the same as AMA-negative PBC.

Case Report

Primary Sclerosing Cholangitis: A Report of Two Liver Explants.: Sun Young Jun, Jung Sun Kim, Eun Sil Yu; Korean J Pathol. 2002;36(6):420-424.

Abstract PDF: Primary sclerosing cholangitis (PSC) is usually progressive to the end stage liver disease and a common indication for liver transplantation (LT). We report two cases of liver explants with PSC. Case 1 was that of a 35-year-old male who was admitted for 4 weeks because he had jaundice. He had been treated several times without receiving a definite diagnosis during the last 7 years because he was excreting dark urine. Case 2 was that of a 30-year-old female who had taken a prednisolone for 6 years for treatment of ulcerative colitis. She also had stones and underwent a cholecystectomy and choledocholithotomy 2 years ago. She had complained of epigastric pain and febrile sensation for 2 months. In both cases, serum levels of aspartate aminotransferase, alanine aminotransferase, alkaline phospatase, gamma glutamyl transpeptidase, and direct builirubin were elevated. Autoantibodies were not detected. Computerized tomography scan and endoscopic retrograde cholangiopancreatography revealed hepato-splenomegaly, stricture of intra- and extra-hepatic bile ducts, and beaded dilatation of the intrahepatic bile ducts. Despite stent insertions and medication, another episode of jaundice occurred in case 1 and cirrhosis finally developed in case 2. The histopathologic features of liver needle biopsy specimens were nonspecific, while explant livers displayed periductal fibrosis, ductopenia, and/or ductular proliferation, which are characteristic features for PSC.

Original Article

Primary Biliary Cirrhosis: A Clinicopathologic Analysis of Four Cases.: Mi Kyung Kim, Yong Il Kim, Hyo Suck Lee, Chung Yong Kim; Korean J Pathol. 1991;25(4):318-326.

Abstract PDF: Primary biliary cirrhosis, a chronic, progressive, and often fatal cholestatic liver disease, is characterized by destruction of intrahepatic bile ducts, portal inflammation and scarring, and the eventual development of cirrhosis and liver failure. We reviewed four cases of primary biliary cirrhosis primarily suspected with peritoneoscopic liver biopsy specimens. All patients were female and their age ranged from 29 to 50 years(median age: 43.8 years). Presenting symptoms were typically pruritus and/or upper abdominal discomfort. On physical examination, interus was presented in 3; hepatomegaly in 3; chronic liver disease stigmata in one. Co-existing autoimmune diseases such as Hashimoto' thyroiditis and Sjoegren' syndrome were found in 2 cases. Biochemical tests of liver function revealed a cholestatic pattern. An antimitochondrial-antibody test was positive in 2 out of 3 patients(75%). Increase levels of serum immunoglobulins, especially of IgM, were evident in 3 cases. Histologic staging(Ludwig et al.) disclosed stage I lesion in one, stage III in two, and stage IV in the remaining one.

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