Improving the Lives of Patients with Alpha-1 Antitrypsin Deficiency

Figures & data

Figure 1 Exacerbations have a negative impact on QoL and lung function in patients with AATD. (A) Box and whisker plot showing SGRQ total scores at 12 months in AATD patients with no, infrequent and frequent AATD exacerbations.⁷ (B) The relationship between change in vital capacity and exacerbation frequency in patients with AATD.²⁶ (C) Rationale for early intervention with antibiotic therapy: Total exacerbation length is related to the length of time in delaying treatment.

Notes: Higher SGRQ scores are correlated with lower lung function measurements. (A) Reproduced with permission of the © ERS 2020: European Respiratory Journal 2005 25: 992–1000; DOI: 10.1183/09031936.05.00074704.⁷ (B) Reprinted with permission of the American Thoracic Society. Copyright © 2020 American Thoracic Society. All rights reserved. Dowson et al (2001). Longitudinal changes in physiological, radiological, and health status measurements in α1-antitrypsin deficiency and factors associated with decline. Am J Respir Crit Care Med. 164;1805–1809.²⁶ The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.

Abbreviations: AATD, Alpha-1 Antitrypsin Deficiency; n, Number of Patients; p, Level of Significance; QoL, Quality of Life; r, Pearson Correlation Coefficient; SGRQ, St George’s Respiratory Questionnaire.

Figure 2 Sputum color chart used at Queen Elizabeth Hospital, Birmingham, UK. In symptom-based diary cards, sputum color is graded 1–5. Samples graded 3–5 indicate increasing neutrophilic infiltration and concomitant elastase activity.

Figure 3 Patient factors that may influence implementation of self-administration.

Figure 4 Selecting the right patient for self-administration – expert pulmonologist perspectives on the ideal candidate.

Note: Reproduced with permission of the © ERS 2020. ERJ Open Research 5 (1) 00171-2018; DOI: 10.1183/23120541.00171-2018 Published 10 March 2019.⁸

Figure 5 Treatable traits typical to patients with AATD that may affect approach to pulmonary rehabilitation. Data from Schols et al.⁵¹

Abbreviation: AATD, Alpha-1 Antitrypsin Deficiency.

NeedhamM, StockleyRA. Exacerbations in alpha-1 antitrypsin deficiency. Eur Respir J. 2005;25(6):992–1000. doi:10.1183/09031936.05.0007470415929953

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HorváthI, CanotilhoM, ChlumskýJ, et al. Diagnosis and management of alpha-1 antitrypsin deficiency in Europe: an expert survey. ERJ Open Res. 2019;5(1):1–11. doi:10.1183/23120541.00171-2018

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ScholsAM, FerreiraIM, FranssenFM, et al. Nutritional assessment and therapy in COPD: a European respiratory society statement. Eur Respir J. 2014;44(6):1504. doi:10.1183/09031936.0007091425234804

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