A Study to Assess Safety and PK of Liquid Alpha1-Proteinase Inhibitor (Human) in Treating Alpha1-Antitrypsin Deficiency

Inclusion Criteria:

- Be between 18 and 70 years of age, inclusive

- Have a diagnosis of congenital AATD

- Have a documented total alpha1-PI level < 11 µM. If the total alpha1-PI level has yet
to be documented, a blood draw for total alpha1-PI level will be obtained at the
Screening Visit

- Have a post-bronchodilator Forced expiratory volume in 1 second (FEV1) ≥ 30% and <
80% of predicted and FEV1/forced vital capacity (FVC) < 70%

- If the subject has received alpha1-PI augmentation therapy of any kind, he/she must
be willing to discontinue that treatment at the Week 1 (Baseline) Visit and remain
off any kind of alpha1-PI treatment, other than the investigational products for this
study, while participating in the study

Exclusion Criteria:

- Subject has had a moderate or severe pulmonary exacerbation during the 4 weeks before
the Week 1 (Baseline) Visit

- History of lung or liver transplant

- Any lung surgery during the past 2 years (excluding lung biopsy)

- Liver cirrhosis confirmed by biopsy

- Elevated liver enzymes (aspartate transaminase [AST], alanine aminotransferase [ALT],
and alkaline phosphatase [ALP]) equal to or greater than 2.5 times the upper limit of
normal

- Severe concomitant disease (e.g., congestive heart failure, clinically significant
pulmonary fibrosis, malignant disease [with the exception of skin cancers other than
melanoma], history of acute hypersensitivity pneumonitis reaction, or current chronic
hypersensitivity pneumonitis)

- Females who are pregnant, breastfeeding or, if of child-bearing potential, unwilling
to practice a highly effective method of contraception (oral, injectable or implanted
hormonal methods of contraception, placement of an intrauterine device (IUD) or
intrauterine system (IUS), condom or occlusive cap with spermicidal
foam/gel/film/cream/suppository, male sterilization, or abstinence) throughout the
study

- Known previous infection with or clinical signs and symptoms consistent with current
hepatitis A virus (HAV), hepatitis B virus (HBV), hepatitis C virus (HCV), or human
immunodeficiency virus (HIV) infection

- Smoking during the past 6 months or a positive urine cotinine test at the Screening
Visit that is due to smoking

- Participation in another investigational drug study within one month prior to the
Week 1 (Baseline) Visit

- History of anaphylaxis or severe systemic response to any plasma-derived alpha1-PI
preparation or other blood product(s)

- Use of systemic steroids above a stable dose equivalent to 5 mg/day prednisone
(i.e.,10 mg every 2 days) within the 4 weeks prior to the Week 1 (Baseline) Visit
inhaled steroids are not considered systemic steroids)

- Use of systemic or aerosolized antibiotics for an exacerbation within the 4 weeks
prior to the Week 1 (Baseline) Visit

- Known selective or severe Immunoglobulin A (IgA) deficiency