| Theme |
Primary Biliary Cirrhosis : update 2003 |
| Title |
Primary Biliary Cirrhosis: Definition and Diagnostic Criteria |
| Author |
Takashi Maeda |
First Department of Internal Medicine, Kochi Medical School |
| Author |
Naoaki Akisawa |
First Department of Internal Medicine, Kochi Medical School |
| Author |
Shinji Iwasaki |
First Department of Internal Medicine, Kochi Medical School |
| Author |
Masafumi Ono |
First Department of Internal Medicine, Kochi Medical School |
| Author |
Toshiji Saibara |
First Department of Internal Medicine, Kochi Medical School |
| Author |
Saburo Onishi |
First Department of Internal Medicine, Kochi Medical School |
| [ Summary ] |
Primary biliary cirrhosis(PBC) is a chronic cholestatic liver disease, mainly affecting middle aged women. The etiology remains unknown, but the disease is associated with profound immunological disturbances and this has been related to bile duct destruction. Historically, Ahrens and colleagues established the concept of PBC in 1950. In their definitive modern description, they reported clinical and laboratory studies in PBC patients with "cirrhotic stage" and the disease was not considered to be due to any known cause and in particular not a consequence of extrahepatic biliary obstruction. However, two thirds of newly diagnosed cases, at present, are asymptomatic patients with "early" stage. Accordingly, the terminology of PBC is historically well entrenched but now misleadingly anachronistic. PBC is diagnosed by the presence of high-titer mitochondrial autoantibodies and the characteristic histology with inflammation and obliteration of the intrehepatic bile ducts (chronic non-suppurative destructive cholangitis). The prognosis of asymptomatic PBC is good in general, but symptomatic PBC patients with jaundice have poor prognoses. Based on data from a national survey of Japanese PBC patients, the 5-year survival rates of patients with asymptomatic PBC, PBC with pruritus, and PBC with jaundice at the time of diagnosis were 97, 88, and 53%, respectively. |