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

Background: Alpha-1-antitrypsin deficiency is a common autosomal reces sive disorder associated with early development of emphysema, fiver ci rrhosis, and hepatocellular carcinoma. The aim of the present study wa s to define prognosis and life expectancy in patients with alpha(1)-an titrypsin deficiency with and without chronic liver disease. Methods: After a follow-up of 15 years the estimated life table analysis of mor tality of 160 patients with alpha(1)-antitrypsin deficiency was retros pectively calculated. The survival time was estimated using the Kaplan -Meier survival curves and was compared with the life expectancy of th e 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 10 6 patients with alpha(1)-antitrypsin deficiency without chronic liver disease none had evidence of additional viral infection. Life expectan cy in patients with alpha(1)-antitrypsin deficiency and chronic liver disease was significantly lower than in patients with alpha(1)-antitry psin deficiency without chronic liver disease (p = 0.001). No differen ce in life expectancy in alpha(1)-antitrypsin deficiency without chron ic liver disease was found in comparison with that of the normal popul ation. Conclusions: We suggest that in alpha(1)-antitrypsin deficiency -associated chronic liver disease it is the high viral coinfection rat her than the inborn error of metabolism itself that is responsible for a deterioration of life expectancy or for the poor prognosis of the d isease.