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What percentage of Alphas see symptoms as a result of Alpha-1?

Most adults with PI ZZ α1-AT deficiency are identified by their pulmonary symptoms and show signs and symptoms of chronic obstructive pulmonary disease, with emphysema developing in about 80% to 100% of individuals with that phenotype. This condition is frequently aggravated by cigarette smoking. The emphysema associated with α1-AT deficiency has distinctive features, including early onset (in the fourth or fifth decade of life), predominant involvement of the lung bases, and panacinar pathology. In contrast, individuals with α1-AT-replete emphysema are older, with predominantly apical and centrilobular emphysema.

The prevalence of associated liver disease has probably been underestimated, but 10% to 40% of these adults may have evidence of cirrhosis. The risk of cirrhosis becomes higher with advancing years, particularly in men. In these cases, a man older than 50 years with evidence of cirrhosis, portal hypertension, or hepatocellular carcinoma with no underlying predisposing cause should evoke suspicion of an underlying metabolic defect such as hemochromatosis or α1-AT deficiency. The features of the liver disease appear to be rapidly progressive when diagnosed at this stage, with a high likelihood of death within 4 years of the identification of liver disease.

Alpha1-Antitrypsin Deficiency – Cleveland Clinic