Follows adults with PiMZ alpha-1 antitrypsin deficiency to study lung changes over time
Follows adults with PiMZ alpha-1 antitrypsin deficiency, tracking lung scans and blood markers over time.
Plain-language title generated from the registry record
Registry title: PiMZ Longitudinal Cohort (PiMZ Logic)
Key facts
- Where
- Birmingham, Chicago, Denver, Los Angeles, and New York, United StatesSee the 5 sites
Where in United States
- University of Alabama at BirminghamBirmingham, United StatesNot yet recruiting
- University of ChicagoChicago, United StatesNot yet recruiting
- National Jewish HealthDenver, United StatesNot yet recruiting
- University of California, Los AngelesLos Angeles, United StatesTaking people
- Columbia University Irving Medical CenterNew York, United StatesNot yet recruiting
Questions to ask the study team
- How often do I need to come in, and for how long each time?
- Are travel costs paid?
- Can I keep my own doctor?
- What happens if I want to stop?
Trial details
- Who runs it
- Columbia University
- Trial code
- NCT06505603
- Record updated
- 16 January 2026
- Original description
Alpha-1 Anti-trypsin Deficiency (AATD) is a genetic disease with lung and liver disease presentations. Presentations are variable in the heterozygous population, the most predominant genotype being PiMZ. The purpose of this study in PiMZ heterozygous patients is to examine the density of the lung as measured by chest computed tomography (CT) and determine if existing emphysema predicts changes in the rate of subsequent emphysema or changes in CT, serum or plasma biomarkers of interest. The overarching goal is to develop biomarkers pertinent to the PiMZ patient that can be used in interventional trials since lung function changes do not typically inform disease progression in AATD.Read full original descriptionShow less
Alpha-1 Antitrypsin PiMZ Longitudinal Cohort (PiMZ Logic)
Alpha-1 Anti-trypsin Deficiency (AATD) is a genetic disease with lung and liver disease presentations. Presentations are variable in the heterozygous population, the most predominant genotype being PiMZ. The purpose of this study in PiMZ heterozygous patients is to examine the density of the lung as measured by chest computed tomography (CT) and determine if existing emphysema predicts changes in the rate of subsequent emphysema or changes in CT, serum or plasma biomarkers of interest. The overarching goal is to develop biomarkers pertinent to the PiMZ patient that can be used in interventional trials since lung function changes do not typically inform disease progression in AATD.
Inclusion: Males and females aged 18 years and older; Understand the study procedures, risks, benefits, purpose; Able and willing to comply with the study procedures; Have PiMZ alpha-1 antitrypsin deficiency; Post bronchodilator FEV1 < 80% predicted AND post bronchodilator FEV1/FVC < 70%; Be an existing member of the Alpha-1 Foundation Clinical Cohort (also known as the Alpha-1 Foundation Research Registry); Agree to have the data collected in this study be shared with the Alpha-1 Foundation Research Registry.
Exclusion: Currently receiving intravenous or subcutaneous immunoglobulin for any disease state; Past or present major surgery on the lungs including pneumonectomy or lobectomy. Wedge resections, past segmentectomy, and pleurodesis surgeries are allowed; Previous lung or liver transplantation or currently on the transplant list; Participation in the active treatment arm of a therapeutic clinical trial at baseline visit unless using one of the Alpha-1 augmentation therapies in alternative doses; Current lung, hematologic, or solid organ malignancy other than skin or cervical Stage 1 cancers within the past 3 years; COPD exacerbation or other pulmonary infection within 6 weeks of baseline visit; Allergy to albuterol; Clinically significant bronchiectasis as defined by the investigator. In general, this would exclude patients with chronic infection of the lungs requiring treatment within the past 6 months including non-tuberculous mycobacterial disease, chronic fungal disease, allergic bronchopulmonary aspergillosis, or known colonization of bronchiectasis with pseudomonas or stenotrophomonas species; Any condition that in the opinion of the investigator might adversely influence the study outcome; Patient receiving biologic immunomodulators that will affect the assessment of the serum biomarkers (as determined by the site PI); AATD non-PiMZ status, including carriers; Pregnancy at the time of the screening visit; Inability to lie still in a supine position for 15 minutes during CT acquisition; Inability to perform quality-controlled lung function testing; Decompensated cirrhosis; Current presence of endobronchial coils or valves in the lung; Patient with Automatic Implantable Cardioverter Defibrillator (AICD) and permanent pacemakers (PPM); Patient with pleural catheters.