Overlapping of primary biliary cirrhosis and small duct primary sclerosing cholangitis: first case report.

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are both autoimmune cholestatic liver disease and the association of these two conditions in the same patient is very rare. We report the case of a female patient presenting with a cholestatic liver disease and a panel of autoantibodies specific for PBC, including antibodies to mitochondrial E2-pyruvate dehydrogenase, gp-210 and Sp-100. Beside these findings, the liver biopsy revealed concentric fibrosis of small biliary ducts and the magnetic resonance cholangiography presented no abnormal findings. Diagnosis of small duct PSC/PBC overlapping was done. No description of this association was found in the literature. Clinical and serological features of this unusual finding are discussed.

Is autoimmune hepatitis a frequent finding among HCV patients with intense interface hepatitis?

AIM: To evaluate the overlap of autoimmune hepatitis in hepatitis C virus (HCV)-infected patients with intense interface hepatitis. METHODS: Among 1759 patients with hepatitis C submitted to liver biopsy, 92 (5.2%) presented intense interface hepatitis. These patients were evaluated regarding the presence of antinuclear antibody (ANA), anti-smooth muscle antibody (SMA) and anti-liver/kidney microsomal antibody (LKM-1), levels of gamma-globulin and histological findings related to autoimmune hepatitis (plasma cell infiltrate and presence of rosettes). RESULTS: Among patients with hepatitis C and intense interface hepatitis there was a low prevalence of autoantibodies (ANA = 12%, SMA = 5%, LKM-1 = 0%) and the median gamma-globulin level was within the normal range. Typical histological findings of autoimmune disease were observed in only two cases (2%). After applying the score for diagnosis of autoimmune hepatitis, only one patient was classified with a definitive diagnosis of autoimmune hepatitis. Since overlap with autoimmune hepatitis was not the explanation for the intense necroinflammatory activity in patients with chronic hepatitis C we sought to identify the variables associated with this finding. The presence of intense interface hepatitis was associated with more advanced age, both at the time of infection and at the time of the biopsy, and higher prevalence of blood transfusion and alcohol abuse. CONCLUSION: Although possible, overlap with autoimmune hepatitis is a very rare association in HCV-infected patients with intense interface hepatitis, an unusual presentation which seems to be related to other host variables.

Factors associated with the progression of hepatic fibrosis in end-stage kidney disease patients with hepatitis C virus infection.

BACKGROUND: Few studies have evaluated the histological aspects of hepatitis C virus (HCV) infection in hemodialysis patients and the factors related to the progression of hepatic fibrosis in this population have not been defined. AIM: To evaluate the influence of host-related factors on the fibrosis progression in end-stage renal disease (ESRD) patients with HCV infection. METHODS: HCV-infected ESRD patients who submitted to liver biopsy were included. The fibrosis stages were classified according to METAVIR scoring system. For the identification of factors associated with more advanced liver fibrosis, the patients were classified into two groups: group 1, absence of septal fibrosis (F0-1) and group 2, presence of septal fibrosis (F2-4). Groups 1 and 2 were compared regarding demographic, epidemiological, and laboratory variables and logistic regression analysis was used to identify the variables that were independently associated with the presence of septal fibrosis. RESULTS: A total of 216 ESRD patients (63% men, 44+/-11 years) were included. In the histological analysis, the fibrosis stages were as follows: F0=36%, F1=41%, F2=12%, F3=7, and 4% had cirrhosis (F4). In the logistic regression model, the variables that were independently associated with the presence of septal fibrosis were duration of infection, estimated age at infection, coinfection with HBV and aspartate aminotransferase levels. CONCLUSION: These findings support the importance of obtaining an adequate immune response to HBV vaccination and careful monitoring of liver disease in patients who become infected at an advanced age and/or those presenting elevated aspartate aminotransferase levels, as these are the main factors associated with the presence of septal fibrosis in ESRD patients.