scholarly journals Opinions and Attitudes of Pulmonologists About Augmentation Therapy in Patients with Alpha-1 Antitrypsin Deficiency. A Survey of the EARCO Group

2022 ◽  
Vol Volume 17 ◽  
pp. 53-64
Author(s):  
Timm Greulich ◽  
Anna Albert ◽  
Werner Cassel ◽  
Tobias Boeselt ◽  
Erika Peychev ◽  
...  
Keyword(s):  
Augmentation Therapy ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Management of lung disease in alpha-1 antitrypsin deficiency: what we do and what we do not know

2021 ◽  
Vol 12_suppl ◽  
pp. 204062232110101
Author(s):  
Igor Barjaktarevic ◽  
Michael Campos
Keyword(s):  
Lung Disease ◽  
Treatment Guidelines ◽  
Management Program ◽  
Lifestyle Changes ◽  
Chronic Obstructive ◽  
Augmentation Therapy ◽  
Lung Function Decline ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Management of lung disease in patients with alpha-1 antitrypsin deficiency (AATD) includes both non-pharmacological and pharmacological approaches. Lifestyle changes with avoidance of environmental pollutants, including tobacco smoke, improving exercise levels and nutritional status, all encompassed under a disease management program, are crucial pillars of AATD management. Non-pharmacological therapies follow conventional treatment guidelines for chronic obstructive pulmonary disease. Specific pharmacological treatment consists of administering exogenous alpha-1 antitrypsin (AAT) protein intravenously (augmentation therapy). This intervention raises AAT levels in serum and lung epithelial lining fluid, increases anti-elastase capacity, and decreases several inflammatory mediators in the lung. Radiologically, augmentation therapy reduces lung density loss over time, thus delaying disease progression. The effect of augmentation therapy on other lung-related outcomes, such as exacerbation frequency/length, quality of life, lung function decline, and mortality, are less clear and questions regarding dose optimization or route of administration are still debatable. This review discusses the rationale and available evidence for these interventions in AATD.

Augmentation therapy of alpha-1 antitrypsin deficiency associated emphysema

2015 ◽  
Vol 32 (4) ◽  
pp. 435-446 ◽  
Author(s):  
J. Traclet ◽  
P. Delaval ◽  
P. Terrioux ◽  
J.-F. Mornex
Keyword(s):  
Augmentation Therapy ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Augmentation therapy for emphysema due to alpha-1-antitrypsin deficiency

2008 ◽  
Vol 2 (1) ◽  
pp. 13-21 ◽  
Author(s):  
Gema Tirado-Conde ◽  
Beatriz Lara ◽  
Marc Miravitlles
Keyword(s):  
Augmentation Therapy ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Augmentation therapy in alpha-1 antitrypsin deficiency: advances and controversies

2010 ◽  
Vol 4 (5) ◽  
pp. 289-312 ◽  
Author(s):  
Adriano R. Tonelli ◽  
Mark L. Brantly
Keyword(s):  
Augmentation Therapy ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Alpha-1-antitrypsin augmentation therapy for alpha-1-antitrypsin deficiency

1988 ◽  
Vol 84 ◽  
pp. 52-62 ◽  
Author(s):  
Richard C. Hubbard ◽  
Ronald G. Crystal
Keyword(s):  
Augmentation Therapy ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Impact of a Health Management Program on Healthcare Outcomes among Patients on Augmentation Therapy for Alpha 1-Antitrypsin Deficiency: An Insurance Claims Analysis

2018 ◽  
Vol 35 (4) ◽  
pp. 467-481 ◽  
Author(s):  
Michael A. Campos ◽  
Michael C. Runken ◽  
Angela M. Davis ◽  
Michael P. Johnson ◽  
Glenda A. Stone ◽  
...  
Keyword(s):  
Health Management ◽  
Management Program ◽  
Healthcare Outcomes ◽  
Augmentation Therapy ◽  
Insurance Claims ◽  
Claims Analysis ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin
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