[Primary biliary cirrhosis: 12-year experience in observation]

To ascertain initial signs of primary biliary cirrhosis (PBC), criteria of its early diagnosis, variants of the course, clinical picture and life quality of PBC patients.

128 PBC patients were examined in the Central Research Institute of Gastroenterology from 1991 to 2002. Quality of life was assessed by SF-36 questionnaire adapted for patients with chronic cholestatic diseases of the liver.

The patients complained of skin puritus (80%), jaundice (66%), fatigue (68.8%), weight loss (24.6%). The disease manifested with skin pruritus in 75.8%, jaundice--in 12.5% patients. Decompensated hepatic cirrhosis (Child-Pugh C) at first hospitalisation was diagnosed in 11.7% cases. The earliest and most significant biochemical markers of PBS were high activity of gammaglutamil-transpeptidase (GGTP) and alkaline phosphatase (AP). IgM level was high in 70.5% cases. Antimitochondrial antibodies were detected in 83.6% examinees. Overlapping with autoimmune hepatitis was seen in 9.4%. One-third of the patients had systemic symptoms. Quality of life deteriorated.

PBC may be long asymptomatic. The absence of skin pruritus is not a sure sign of the disease absence. To detect PBC early, young and middle-aged women with unexplained weakness or skin pruritus should undergo a comprehensive examination including measurements of GGTP, AP, AMA, IgM, morphological investigation of liver tissue. PBC patients had low quality of life. This should be accounted for when treating patients, in determination of their working ability and for early referral for liver transplantation.