| Theme |
PSC and PBC -- Recent Progress in Diagnosis and Treatment |
| Title |
Histopathological Findings of Primary Biliary Cirrhosis |
| Author |
Yuko Kakuda |
Department of Pathology, Shizuoka Cancer Center / Department of Human Pathology, Kanazawa University Graduate School of Medicine |
| Author |
Kenichi Harada |
Department of Human Pathology, Kanazawa University Graduate School of Medicine |
| Author |
Yasuni Nakanuma |
Department of Pathology, Shizuoka Cancer Center / Department of Human Pathology, Kanazawa University Graduate School of Medicine |
| [ Summary ] |
In terms of histology, the most characteristic and indicative diagnostic finding for primary biliary cirrhosis (PBC) is chronic nonsupprative destructive cholangitis, affecting the interlobular bile ducts. After the onset of cholangiopathy, the affected bile ducts finally disappear, resulting in chronic cholestasis, and development of liver fibrosis. In the new histological staging and grading system for primary biliary cirrhosis we proposed, staging is accomplished on the basis of degree of liver fibrosis, loss of interlobular bile ducts, and deposition of orcein-positive granules representing chronic cholestasis. In terms of grading, degrees of chronic cholangitis and hepatitis activities are evaluated individually. These histological components are fundamental lesions being used to diagnose and predict PBC patientsʼ outcome. In addition there are many findings caused by chronic cholestasis or immune response in PBC patients. It is important to evaluate whether nodular regenerative hyperplasia, reflecting portal hypertension, is present. A liver biopsy still remains necessary not only to diagnose PBC in certain cases such as antimitochondrial antibody negative patients, but also to accurately evaluate PBC patientsʼ risks of disease progression. |