PH 3/4 GLASSIA SAFETY, IMMUNOGENICITY, AND BRONCHOALVEOLAR LAVAGE STUDY

Inclusion Criteria:

1. Male or female participants meeting the following age criteria:

1. For participants who will undergo bronchoscopy/ bronchoalveolar lavage (BAL)
procedures: 18 to 75 years of age at the time of screening.

2. For participants who will be waived from undergoing bronchoscopy/BAL procedures:
18 years of age or older at the time of screening.

2. Documented Alpha1-Proteinase Inhibitor (A1PI) genotype of Pi*Z/Z, Pi*Z/Null,
Pi*Malton/Z, Pi*Null/Null, or other "at-risk" allelic combinations such as SZ
(excluding MS and MZ) and an endogenous A1PI plasma levels of ≤11 μM.

3. Screening levels of endogenous plasma (antigenic) A1PI of ≤11 μM may be collected at
any time during the screening period for treatment-naïve participants, or following a
4 week minimum wash-out from previous augmentation therapy in treatment-experienced
participants.

4. Participants must have at least one of the following: clinical diagnosis of emphysema,
evidence of emphysema on computerized tomography (CT) scan of the chest, and/or
evidence of airway obstruction which is not completely reversed with bronchodilator
treatment at the time of screening.

5. If the participant is being treated with any respiratory medications including inhaled
bronchodilators, inhaled anticholinergics, inhaled corticosteroids, or low-dose
systemic corticosteroids (prednisone ≤10 mg/day or its equivalent), the doses of the
participant's medications have remained unchanged for at least 14 days prior to
screening.

6. The participant is a nonsmoker or has ceased smoking for a minimum of 13 weeks prior
to screening (serum cotinine level at screening within normal range of a nonsmoker)
and agrees to refrain from smoking throughout the course of the study. Participants
with a positive cotinine test due to nicotine replacement therapy (eg, patches,
chewing gum), vapor cigarettes, or snuff are eligible.

7. If female of childbearing potential, the participant presents with a negative
pregnancy test at screening and agrees to employ adequate birth control measures for
the duration of the study.

8. The participant is willing and able to comply with the requirements of the protocol.

9. The participant must have pulmonary function at the time of screening meeting both of
the following:

1. Post-bronchodilator forced expiratory volume in 1 second (FEV1) ≥50% of
predicted.

2. If FEV1 is >80% predicted, then FEV1/forced vital capacity (FVC) must be <0.7.
*Note: Inclusion criterion #1a and #9a are not applicable to participants who are
not required to undergo the bronchoscopy/BAL procedures.

Exclusion Criteria:

1. The participant is experiencing or has a history of clinically significant pulmonary
disease (other than chronic obstructive pulmonary disease (COPD), emphysema, chronic
bronchitis, mild bronchiectasis, and stable asthma).

2. The participant is experiencing or has a history of chronic severe cor pulmonale
(resting mean pulmonary artery pressure ≥40 millimeter(s) of mercury (mm Hg)).

3. The participant routinely produces more than 1 tablespoon of sputum per day.

4. The participant has a history of frequent pulmonary exacerbations (greater than 2
moderate or severe exacerbations within 52 weeks prior to screening.

5. The participant is experiencing a pulmonary exacerbation at the time of screening
(participant may be re-screened 4 weeks after the clinical resolution of an
exacerbation).

6. The participant has clinically significant abnormalities (other than emphysema,
chronic bronchitis, or mild bronchiectasis) detected on chest X-ray or CT scan at the
time of screening. (Past records obtained within 52 weeks prior to screening may be
used, if available.)

7. The participant has clinically significant abnormalities detected on a 12-lead
electrocardiogram (ECG) performed at the time of screening. (Past records obtained
within 26 weeks prior to screening may be used, if available.)

8. The participant has clinically significant congestive heart failure with New York
Heart Association (NYHA) Class III/IV symptoms.

9. The participant is experiencing an active malignancy or has a history of malignancy
within 5 years prior to screening, with the exception of the following: adequately
treated basal cell or squamous cell carcinoma of the skin, carcinoma in situ of the
cervix, or stable prostate cancer not requiring treatment.

10. The participant has a history of lung or other organ transplant, is currently on a
transplant list, or has undergone major lung surgery.

11. The participant is receiving long-term around-the-clock oxygen supplementation. (The
following are allowed: short-term use of oxygen supplementation [eg, for the
management of acute COPD exacerbation], oxygen supplementation required during night
time only, and supplemental oxygen (O2) with continuous positive airway pressure
[CPAP] or bi-level positive airway pressure [BiPAP]).

12. Known history of hypersensitivity following infusions of human blood or blood
components.

13. Immunoglobulin A (IgA) deficiency (<8 mg/dL at screening).

14. Abnormal clinical laboratory results obtained at the time of screening meeting any of
the following criteria:

1. Serum alanine aminotransferase (ALT) >3.0 times upper limit of normal (ULN)

2. Serum total bilirubin >2.0 times ULN

3. >2+proteinuria on urine dipstick analysis

4. Serum creatinine >2.0 times ULN

5. Absolute neutrophil count (ANC) <1500 cells/mm^3

6. Hemoglobin (Hgb) <9.0 g/dL

7. Platelet count <100,000/mm^3

15. Ongoing active infection with hepatitis A virus (HAV), hepatitis B virus (HBV),
hepatitis C virus (HCV), or human immunodeficiency virus (HIV) Type 1 or 2 infection
at the time of screening.

16. The participant has any clinically significant medical, psychiatric, or cognitive
illness, or any other uncontrolled medical condition (eg, unstable angina, transient
ischemic attack) that, in the opinion of the investigator, would impede the
participant's ability to comply with the study procedures, pose increased risk to the
participant's safety, or confound the interpretation of study results.

17. The participant has participated in another clinical study involving an
investigational product or investigational device within 30 days prior to enrollment
or is scheduled to participate in another clinical study involving an investigational
product or device during the course of this study.

18. The participant is a family member or employee of the investigator.

19. If female, the participant is nursing at the time of screening.

20. The participant has contraindication(s) to bronchoscopy such as recent myocardial
infarction, unstable angina, other cardiopulmonary instability, tracheal obstruction
or stenosis, moderate to severe hypoxemia or any degree of hypercapnia, unstable
asthma, Stage 4 or 5 chronic kidney disease, pulmonary hypertension, severe
hemorrhagic diathesis, and cervical C1/C2 arthritis.

21. The participant has had lung surgery which may interfere with bronchoscopy.

22. Known history of allergic/hypersensitivity reactions to medications used during and
for perioperative care associated with the bronchoscopy/BAL procedures, such as local
anesthetics, sedatives, pain control medications.

23. The participant is receiving or requires long-term (>4 weeks) immunosuppressive
therapy, such as systemic corticosteroids at doses greater than 10 mg/day of
prednisone (or its equivalent), mycophenolate mofetil, azathioprine, cyclophosphamide,
and rituximab.

24. If a participant is receiving anticoagulant or anti-platelet therapy (such as warfarin
and clopidogrel), the participant is unwilling to or unable to safely discontinue
anticoagulant or anti-platelet therapy within 7 days prior to until at least 24 hours
after the BAL procedures. An exception is low-dose aspirin alone which is allowed.

- Note: Exclusion criteria #20, #21, #22, #23, and #24 are not applicable to
participants who are not required to undergo the bronchoscopy/BAL procedures.