Alpha-1 is the #1 known genetic risk factor for COPD1

Alpha-1 may be a contributing cause in approximately 3 in 10 cases of COPD in the United States2

Alpha1-antitrypsin (AAT) deficiency, also called alpha-1 or genetic COPD, is a relatively common but widely underdiagnosed inherited condition that increases the risk of early onset COPD and of liver disease.2-4 AAT deficiency is the most common genetic risk factor for COPD, a highly prevalent, debilitating lung condition.5,6

Survival analysis of individuals with severe alpha-1 (Pi ZZ)
vs total US population8

A landmark study indicated that severe alpha-1 significantly shortened life span.8
Mean survival of the alpha-1 cohort (n=120) was 16% at 60 years of age, compared with 85% for the general population.8

Used with permission of the American Thoracic Society. Copyright 2012, American Thoracic Society. Study Design, 1988: All 120 Pi ZZ subjects evaluated as inpatients in NIH Clinical Center. Various demographic features, clinical findings, and electrocardiogram evaluations were obtained from medical records. Blood and urine laboratory findings were obtained through computerized archives of the Clinical Center Laboratories. Chest radiographs and scintigraphy scans were evaluated de novo at the time of this analysis, with knowledge of the diagnosis but without knowledge of any of the other data. Lung function tests were obtained from the computerized files of the Pulmonary Branch. Mortality data were obtained at the time of this analysis. Alpha-1 antitrypsin protein phenotypes were determined by a combination of isoelectric focusing of serum, quantitation of AAT levels in serum, and family studies.8

Alpha-1 is not rare, but is rarely diagnosed9

Smokers and ex-smokers make up a large part of the diagnosed alpha-1 population, but it occurs in both smokers and nonsmokers.1

FEV1 Percentage predicated by age and distinguished by smoking status10*

*378 Pi ZZ patients, †Stage III severe COPD, GOLD guidelines

Approximately 90% of patients living
with alpha-1 remain undiagnosed11

These major respiratory organizations recommend screening all patients with COPD or treatment-resistant asthma2,12-15

AlphaIDTM is a comprehensive program designed to rule out alpha-1 in patients with COPD

Identify patients at risk with fast, reliable results through AlphaID. AlphaID is provided by Grifols at no cost and may not be billed to you, your patients, or their insurers.*

Genetic Detection

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No-Cost Testing Program

*The receipt or this free testing service does not create any expectation or obligation to purchase or use any product or service offered by any manufacturer.

An alpha-1 family tree

Model illustrating inheritance patterns determined by two alleles for alpha-116

Inheritance is determined by two alleles, one from each parent. Common alleles are M (normal) and Z (defective). The combination of these alleles (eg, MM, MZ, ZZ) determines the level of AAT protein produced and the risk of disease.16

“The only way to know [if a person has alpha-1] is by screening. You know, [patients with alpha-1] look the same. They walk the same. They cough the same. They have all the same symptoms as your other patients with COPD. The only way to know is by screening.”

Corinne Young Healthcare Provider

“I know now, as an expert in alpha1-antitrypsin deficiency, it's not really rare, it's just rarely thought of, or rarely tested.”

Dr. Jeffrey Miskoff Pulmonologist

Take on alpha-1
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Begin screening with AlphaID™.

AlphaID is free and makes it easy to screen for alpha-1. A simple cheek swab sample provides 100% accurate
and reliable genotyping results.3,17

 

The receipt of this free testing service does not create any expectation or obligation to purchase or use any product or service offered by any manufacturer.

 

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Important Safety Information

PROLASTIN®-C LIQUID is an alpha1-proteinase inhibitor (human) (alpha1-PI) indicated for chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of alpha1-PI (alpha1-antitrypsin deficiency). 


Limitations of Use

  • The effect of augmentation therapy with any alpha1-PI, including PROLASTIN-C LIQUID, on pulmonary exacerbations and on the progression of emphysema in alpha1-PI deficiency has not been conclusively demonstrated in randomized, controlled clinical trials.
  • Clinical data demonstrating the long-term effects of chronic augmentation or maintenance therapy with PROLASTIN-C LIQUID are not available.
  • PROLASTIN-C LIQUID is not indicated as therapy for lung disease in patients in whom severe alpha1-PI deficiency has not been established.

PROLASTIN-C LIQUID is contraindicated in immunoglobulin A (IgA)-deficient patients with antibodies against IgA or patients with a history of anaphylaxis or other severe systemic reaction to alpha1-PI products.

Hypersensitivity reactions, including anaphylaxis, may occur. Monitor vital signs and observe the patient carefully throughout the infusion. If hypersensitivity symptoms occur, promptly stop PROLASTIN-C LIQUID infusion and begin appropriate therapy.

Because PROLASTIN-C LIQUID is made from human plasma, it may carry a risk of transmitting infectious agents, eg, viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. This also applies to unknown or emerging viruses and other pathogens.

The most common adverse reactions during PROLASTIN-C LIQUID clinical trials in >5% of subjects were diarrhea and fatigue, each of which occurred in 2 subjects (6%).

Please see full Prescribing Information for PROLASTIN-C LIQUID.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

References

  1. Han MK, Martinez FJ. Host, gender, and early-life factors as risks for chronic obstructive pulmonary disease. Clin Chest Med. 2020;41(3):329-337.
  2. Campos MA, Wanner A, Zhang G, Sandhaus RA. Trends in the diagnosis of symptomatic patients with the α₁-antitrypsin deficiency between 1968 and 2003. Chest. 2005;128(3):1179–1186.
  3. Stoller JK. Myths and misconceptions about a1-antitrypsin deficiency. Arch Intern Med. 2009;169(6):546-550.
  4. Stoller JK, Aboussouan LS. A review of alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2012;185(3):246-259.5.
  5. Silverman EK. Genetics of COPD. Annu Rev Physiol. 2020 February 2020;82:413-431. doi:10.1146/annurev-physiol-021317-121224.
  6. About COPD. Centers for Disease Control and Prevention. May 15, 2024. Accessed December 11, 2025. https://www.cdc.gov/copd/about/index.html.
  7. Meischl T, Schmid-Scherzer K, Vafai-Tabrizi F, et al. The impact of diagnostic delay on survival in alpha-1 antitrypsin deficiency: results from the Austrian Alpha-1 Lung Registry. Respir Res. 2023;24(1):34.
  8. Brantly ML, Paul LD, Miller BH, Falk RT, Wu M, Crystal RG. Clinical features and history of the destructive lung disease associated with alpha-1 antitrypsin deficiency of adults with pulmonary symptoms. Am Rev Respir Dis. 1988;138(2):327–336.
  9. de Serres FJ. Alpha-1 antitrypsin deficiency is not a rare disease but a disease that is rarely diagnosed. Environ Health Perspect. 2003;111(16):1851–1854.
  10. DeMeo DL, Sandhaus RA, Barker AF, et al. Determinants of airflow obstruction in severe alpha-1 antitrypsin deficiency. Thorax. 2007;62(9):806–813.
  11. Brantly M, Campos M, Davis AM, et al. Detection of alpha-1 antitrypsin deficiency: the past, present and future. Orphanet J Rare Dis. 2020;15(1):96.
  12. Global Initiative for Chronic Obstructive Lung Disease. Pocket Guide to COPD Diagnosis, Management, and Prevention. 2022:1–48.
  13. American Thoracic Society/European Respiratory Society. American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003;168(7):818–900.
  14. Sandhaus RA, Turino G, Brantly ML, et al. The diagnosis and management of alpha-1 antitrypsin deficiency in the adult. Chronic Obstr Pulm Dis. 2016;3(3):668–682.
  15. World Health Organization. α₁-antitrypsin deficiency: memorandum from a WHO meeting. Bull World Health Organ. 1997;75(5):397–415.
  16. Alpha-1 Antitrypsin Deficiency Healthcare Provider’s Guide. Alpha-1 Foundation. Version 3.1, September 2020.
  17. A1AT Genotyping Assay Package Insert. Progenika Biopharma.