Treatment of liver disease in alpha-1

Liver disease due to alpha-1-antitrypsin (A1AT) deficiency (also known as alpha-1) is treated using the same general supportive measures as other types of liver disease. The goal is to lessen the impact of complications (eg, cirrhosis) or prevent them altogether.¹

• In cases where the liver disease becomes life-threatening, liver transplantation has become common.
  • Assuming that the donor does not have alpha-1 (eg, has a Pi MM genotype), the transplant will also resolve the alpha-1 and serum levels of A1AT will return to the normal range.
• Most patients with a Pi ZZ genotype are followed annually by a liver specialist, but management is typically conservative in the absence of signs or symptoms of liver disease.