International Study Evaluating the Safety and Efficacy of Inhaled, Human, Alpha-1 Antitrypsin (AAT) in Alpha-1 Antitrypsin Deficient Patients With Emphysema

Principal Inclusion Criteria: * Diagnosis of emphysema confirmed by CT scan. If a report of past CT scan is not available at site documenting then a CT scan is to be performed at screening * Male or female patients at least 18 years of age. * Able and willing to sign an informed consent. * Patient with record of congenital AAT deficiency of phenotype PiZZ (homozygote) or other rare phenotypes related to AAT deficiency and with AAT serum level ≤ 11 micromole. For patients receiving IV AAT augmentation therapy the serum AAT level threshold does not apply. * FEV1/SVC \<70% of predicted value post bronchodilator (SVC is slow VC) and FEV1 \< 80% of predicted value post-bronchodilator * History of at least two moderate or severe exacerbations that required change in treatment (antibiotics, systemic steroids, hospitalization) in the last 18 months prior to date of screening , with at least one of these occurring within the last 12 months prior to screening. * Ability to comply with completion of electronic diary. * Ability to self-administer inhaled AAT. * No significant abnormalities in serum hematology, serum chemistry and serum inflammatory / immunogenic markers according to the Principal Investigator's judgment, taking into considerations the potential effects of the AAT deficiency. * No significant abnormalities in urinalysis according to the Principal Investigator's judgment, taking into considerations the potential effects of the AAT deficiency. * No significant abnormalities in ECG per investigator judgment. * Negative for HBsAg and for antibodies to HCV, HIV-1. * AAT deficient patients who are either naïve (not receiving IV augmentation therapy) or AAT deficient patients (receiving IV augmentation therapy), if they have been stable on regular therapy for at least 3 months prior to the screening visit and are willing to continue the same regime throughout this trial. Note that only sites in Germany can recruit patients who are currently being treated with IV AAT.Patients who stopped IV augmentation treatment 6 months prior to screening date and will not re-start this treatment for the course of the study will be considered Naïve. * Non-pregnant, non-lactating female patients, whose screening pregnancy test is negative and who are using contraceptive methods deemed reliable by the investigator, or who are at least 2 years post-menopausal or surgically sterilized. Principal Exclusion Criteria: * FEV1 \>= 80% or FEV1 \< 20% of predicted value post-bronchodilator. * FEV1/SVC\>=70% * History of lung transplant. * Any lung surgery within the past two years. * On any thoracic surgery waiting list. * End of last exacerbation less than 6 weeks prior to screening/re-screening visit. * Clinically significant intercurrent illnesses (except for respiratory or liver disease secondary to AAT deficiency), including: cardiac, hepatic, renal, endocrine, neurological, hematological, neoplastic, immunological, skeletal or other) that in the opinion of the investigator, could interfere with the safety, compliance or other aspects of this study. Patients with well-controlled, chronic diseases could possibly be included after consultation with the treating physician and the sponsor. * Active smoking during the last 12 months from screening date. * Pregnancy or lactation. * Woman of child-bearing potential not taking adequate contraception deemed reliable by the investigator. * Presence of psychiatric/ mental disorder or any other medical disorder which might impair the patient's ability to give informed consent or to comply with the requirements of the study protocol. * Evidence of ongoing viral infection with HCV, HBV and/or HIV. * Evidence of alcohol abuse or history of alcohol abuse or illegal and/or legally prescribed drugs. * IgA Deficiency * History of life threatening allergy, anaphylactic reaction, or systemic response to human plasma derived products. * Participation in another clinical trial within 30 days prior to baseline visit. * Inability to attend scheduled clinic visits and/or comply with the study protocol. * Any other factor that, in the opinion of the investigator, would prevent the patient from complying with the requirements of the protocol.