PROGNOSIS AND LIFE EXPECTANCY IN ALPHA-1-ANTITRYPSIN DEFICIENCY AND CHRONIC LIVER-DISEASE

Background: Alpha-1-antitrypsin deficiency is a common autosomal recessive disorder associated with early development of emphysema, fiver cirrhosis, and hepatocellular carcinoma. The aim of the present study was to define prognosis and life expectancy in patients with alpha(1)-antitrypsin deficiency with and without chronic liver disease. Methods: After a follow-up of 15 years the estimated life table analysis of mortality of 160 patients with alpha(1)-antitrypsin deficiency was retrospectively calculated. The survival time was estimated using the Kaplan-Meier survival curves and was compared with the life expectancy of the age- and sex-matched population of west Austria. Results: Fifty-four patients with alpha(1)-antitrypsin deficiency had evidence of chronic liver disease; of these, 78% showed positive viral markers. Of the 106 patients with alpha(1)-antitrypsin deficiency without chronic liver disease none had evidence of additional viral infection. Life expectancy in patients with alpha(1)-antitrypsin deficiency and chronic liver disease was significantly lower than in patients with alpha(1)-antitrypsin deficiency without chronic liver disease (p = 0.001). No difference in life expectancy in alpha(1)-antitrypsin deficiency without chronic liver disease was found in comparison with that of the normal population. Conclusions: We suggest that in alpha(1)-antitrypsin deficiency-associated chronic liver disease it is the high viral coinfection rather than the inborn error of metabolism itself that is responsible for a deterioration of life expectancy or for the poor prognosis of the disease.