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An analysis of the degree of concordance among international guidelines regarding alpha-1 antitrypsin deficiency

Authors Attaway A, Majumdar U, Sandhaus RA, Nowacki AS, Stoller JK

Received 13 March 2019

Accepted for publication 1 August 2019

Published 5 September 2019 Volume 2019:14 Pages 2089—2101


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 3

Editor who approved publication: Dr Richard Russell

Amy Attaway,1,2 Uddalak Majumdar,1,3 Robert A Sandhaus,4,5 Amy S Nowacki,6 James K Stoller2,7

1Cleveland Clinic Lerner School of Medicine, Cleveland, OH, USA; 2Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA; 3Department of Hospital Medicine, Cleveland Clinic, Cleveland, OH, USA; 4Alpha-1 Antitrypsin Deficiency Program, National Jewish Health, Denver, CO, USA; 5Alpha-1 Foundation, Coral Gables, FL, USA; 6Department of Quantitative Health Sciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA; 7Education Institute, Cleveland Clinic, Cleveland, OH, USA

Correspondence: Amy Attaway
Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
Tel +1 216 445 2807

Background: Practice guidelines (PGs) attempt to standardize practice to optimize care. For uncommon lung diseases like alpha-1 antitrypsin deficiency (AATD), a paucity of definitive studies and geographic variation in prevalence may hamper guideline generation. The current study assembled and assesses the degree of concordance among available PGs regarding AATD.
Methods: To assess concordance, 15 eligible guidelines focused on AATD were evaluated regarding recommendations surrounding 24 key clinical issues. A Delphi process achieved consensus on ratings for each statement among 3 reviewers. Agreement was quantified as the proportion of guideline comparisons with a matching rating.
Results: The overall level of agreement was 47% (1190/2520 comparisons). The overall “affirmative agreement percentage” (ie, when guidelines agreed in endorsing a practice), was 42% (501/1190 comparisons). The agreement for individual clinical statements ranged from 26% to 75%. A broad consensus was seen in the recommendation to test all patients with a history of fixed obstruction on pulmonary function testing (either from asthma or COPD). Given that AATD is an under-recognized disease and that diagnosis often occurs at a late stage, the authors are encouraged by this consensus. Where overall the guidelines were less explicit was when to refer to a specialist or AATD center. Deciding on a treatment strategy requires a thorough understanding of the alpha 1 serum level, genotype, pulmonary function testing, and imaging, and therefore the authors feel that all patients would benefit from a specialty referral if the diagnosis of AATD is being considered.
Conclusion: Available guidelines regarding AATD frequently disagreed in management recommendations. Possible explanations for discordance include differences in regional prevalence, availability of augmentation therapy, and insurance environments. Attempts to harmonize the various guidelines by empaneling a broadly representative international group of disease experts should be considered for AATD. Similar comparisons among guidelines for other diseases are recommended.

Keywords: alpha-1 antitrypsin deficiency, practice guidelines, chronic obstructive pulmonary disease, clinical management

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