Abstract
The patient was a 48-year-old woman, whose earliest symptoms were generalized pruritus and skin pigmentation, and was admitted to our university hospital in November, 1971. The results of liver function tests on admission showed a cholestasis with markedly elevated alkaline phosphatase and cholesterol levels and with strongly positive antimitochondrial antibody. Histological examination of a surgically biopsied specimen of the liver revealed findings of chronic non-suppurative destructive cholangitis. Various kinds of therapy were tried without beneficial effects except for plasmapheresis, which was aimed at the relief of the xanthomatous neuropathy of the hands with a resultant improvement of symptoms, decreased serum cholesterol level and reduction of xanthoma.
In autopsy, about 6 years after the onset, the liver was cirrhotic and 850g in weight, and the result of histological examination was compatible with the diagnosis of primary biliary cirrhosis.