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

{"article_title":"An analysis of the degree of concordance among international guidelines regarding alpha-1 antitrypsin deficiency.","author":"Attaway A, Majumdar U, Sandhaus RA, Nowacki AS, Stoller JK","journal_title":"International journal of chronic obstructive pulmonary disease","issn":"1178-2005","isbn":"","publication_date":"2019 Sep 05","volume":"14","issue":"","first_page":"2089","page_count":"","accession_number":"31564856","doi":"10.2147\/COPD.S208591","publisher":"DOVE Medical Press","doctype":"Comparative Study","subjects":"Healthcare Disparities standards; Practice Guidelines as Topic standards; Practice Patterns, Physicians' standards; alpha 1-Antitrypsin Deficiency therapy; Consensus; Delphi Technique; Humans; alpha 1-Antitrypsin Deficiency diagnosis; alpha 1-Antitrypsin Deficiency epidemiology; alpha 1-Antitrypsin Deficiency physiopathology","interest_area":["Pulmonology"," Internal\/Family Medicine"],"abstract":"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. Competing Interests: Dr Robert A Sandhaus served in Advisory Board for Grifols, CSL Behring, and Shire, outside the submitted work. The authors report no other conflicts of interest in this work. \ufffd 2019 Attaway et al.","url":"https:\/\/search.ebscohost.com\/login.aspx?direct=true&db=mdl&AN=31564856&authtype=shib&custid=ns346513","isPdfLink":true,"isSAML":false,"additionalInfo":{"Authored_By":"Attaway A, Majumdar U, Sandhaus RA, Nowacki AS, Stoller JK","Journal_Info":"Publisher: DOVE Medical Press Country of Publication: New Zealand NLM ID: 101273481 Publication Model: eCollection Cited Medium: Internet ISSN: 1178-2005 (Electronic) Linking ISSN: 11769106 NLM ISO Abbreviation: Int J Chron Obstruct Pulmon Dis Subsets: MEDLINE","Publication_Type":"Comparative Study; Journal Article; Systematic Review","Published_Date":"2019-09-05","Source":"International journal of chronic obstructive pulmonary disease [Int J Chron Obstruct Pulmon Dis] 2019 Sep 05; Vol. 14, pp. 2089-2101. Date of Electronic Publication: 2019 Sep 05 (Print Publication: 2019).","Languages":"English","Electronic_ISSN":"1178-2005","MeSH_Terms":"Healthcare Disparities\/*standards , Practice Guidelines as Topic\/*standards , Practice Patterns, Physicians'\/*standards , alpha 1-Antitrypsin Deficiency\/*therapy, Consensus ; Delphi Technique ; Humans ; alpha 1-Antitrypsin Deficiency\/diagnosis ; alpha 1-Antitrypsin Deficiency\/epidemiology ; alpha 1-Antitrypsin Deficiency\/physiopathology","Subjects":"Consensus, Delphi Technique, Humans, alpha 1-Antitrypsin Deficiency diagnosis, alpha 1-Antitrypsin Deficiency epidemiology, alpha 1-Antitrypsin Deficiency physiopathology, Healthcare Disparities standards, Practice Guidelines as Topic standards, Practice Patterns, Physicians' standards, alpha 1-Antitrypsin Deficiency therapy","Title_Abbreviations":"International journal of chronic obstructive pulmonary disease","Volume":"14"},"header":{"DbId":"mdl","DbLabel":"MEDLINE Ultimate","An":"31564856","RelevancyScore":"836","PubType":"Academic Journal","PubTypeId":"academicJournal","PreciseRelevancyScore":"836.128234863281"},"plink":"https:\/\/search.ebscohost.com\/login.aspx?direct=true&site=eds-live&db=mdl&AN=31564856&authtype=shib&custid=ns346513&group=main&profile=eds","upload_link":"https:\/\/search.ebscohost.com\/login.aspx?direct=true&site=eds-live&db=mdl&AN=31564856&authtype=shib&custid=ns346513&group=main&profile=eds"}