Recurrence of primary biliary cirrhosis and development of autoimmune hepatitis after liver transplant: A blind histologic study.

AIM: This long-term study aimed to evaluate recurrence and evolution of primary biliary cirrhosis (PBC) after orthotopic liver transplantation (OLT). METHODS: We reviewed "blindly" allograft biopsy specimens of women who underwent transplantation for PBC (n = 84), and women who received a transplant for chronic hepatitis C virus infection (CHCV ) (n = 108). All needle liver biopsy specimens obtained more than 6 months post-OLT were examined, including 83 specimens from 44 PBC patients and 152 specimens from 58 CHCV patients. RESULTS: Granulomatous destructive cholangitis was found in five biopsies from four PBC patients (P = 0.0048). Non-necrotizing epithelioid cell granulomas were present in four biopsies from four PBC patients, and in two biopsies from one CHCV patient. Piecemeal necrosis (P = 0.0002), lobular necroinflammatory activity (P < 0.0001), steatosis (P < 0.0001) and fibrosis (P < 0.0001) were more prevalent in CHCV patients than PBC patients. Four PBC patients developed histologic evidence of autoimmune hepatitis (AIH), at a mean time of 3.66 years post-OLT. One of these patients had histologic features of AIH/PBC overlap syndrome. All four patients developed bridging fibrosis (n = 2) or cirrhosis (n = 2). No other PBC patient had evidence of cirrhosis after OLT. CONCLUSIONS: Histologic findings indicative of recurrent PBC were present in 15.9% of the PBC patients undergoing biopsy in this series. However, this group of patients did not suffer significant bile duct loss or fibrosis, as compared to the control group, suggesting that recurrent PBC is a mild or slowly progressive disease. Histologic evidence of AIH was observed in allograft biopsies of some PBC patients.

Importance of specimen size in accurate needle liver biopsy evaluation of patients with chronic hepatitis C.

BACKGROUND & AIMS: In patients with chronic hepatitis C (CHC), percutaneous needle liver biopsy examination establishes the severity of necroinflammatory activity and fibrosis, thus guiding treatment decisions. Optimal biopsy specimen size remains controversial. We sought to determine how varying lengths of biopsy specimens influence the grading and staging of CHC. METHODS: We used 100 liver biopsy specimens from patients with CHC. The slides were evaluated blindly using the METAVIR scoring system, after being covered with paper, so that only specific specimen lengths (5 mm, 10 mm, 15 mm, and > or =20 mm) were visible. In each case, the scores obtained with biopsies 5 mm, 10 mm, or 15 mm long were compared with the scores at 20 mm or greater by weighted kappa statistics (kappa of >.75 signified excellent agreement). A subset of specimens 20 mm or greater was selected for a blinded repeat scoring to assess intraobserver agreement. The kappa statistics for the designated features and lengths were compared using analysis of variance. RESULTS: In assessing the stage of fibrosis, the weighted kappa statistics for agreement with the 20-mm or greater score at 5 mm, 10 mm, and 15 mm were .75, .85, and .92, respectively. In assessing the histologic activity score, the corresponding figures were .73, .81, and .77, respectively. Average kappa statistic comparisons showed that intraobserver agreement was significantly better than agreement between the 20-mm or greater scores and those at shorter lengths; the 5-mm kappa scores were significantly lower than the others; and there was no significant difference between the 10-mm and 15-mm kappa scores. CONCLUSIONS: Liver biopsy specimens measuring at least 10 mm usually reflect the grade and stage of CHC reliably. Relatively little improvement in diagnostic accuracy is obtained with longer specimens.

Autoimmune hyperlipidemia in a child with autoimmune hepatitis.

The first reported case of a girl with a combination of autoimmune hyperlipidemia and autoimmune hepatitis is described. She presented at the age of 9 years with fever, headaches, and abnormal lipid profile. Months later, she had clinical manifestations, biochemical findings, and the histologic picture of autoimmune hepatitis. Subsequently, she also showed signs and symptoms of systemic lupus erythematosus. All of her clinical manifestations and biochemical abnormalities dramatically improved with immunosuppression. The overlapping syndrome of systemic lupus erythematosus, autoimmune hepatitis, and autoimmune hyperlipidemia is discussed.