Please use this identifier to cite or link to this item: doi:10.22028/D291-37461
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Title: Deterioration of quality of life is associated with the exacerbation frequency in individuals with alpha-1-antitrypsin deficiency - analysis from the German Registry
Author(s): Bernhard, Nikolas
Lepper, Philipp M.
Vogelmeier, Claus
Seibert, Martina
Wagenpfeil, Stefan
Bals, Robert
Fähndrich, Sebastian
Language: English
Title: International Journal of Chronic Obstructive Pulmonary Disease
Volume: 12
Pages: 1427-1437
Publisher/Platform: DOVE
Year of Publication: 2017
Free key words: SGRQ
quality of life
alpha-1-antitrypsin deficiency
DDC notations: 610 Medicine and health
Publikation type: Journal Article
Abstract: Background: Alpha-1-antitrypsin deficiency (AATD) is a rare hereditary disease that is associated with a higher risk to develop chronic obstructive pulmonary disease and liver cirrhosis. Previous cross-sectional studies on AATD individuals have shown a relationship between worse St George’s Respiratory Questionnaire (SGRQ) scores and elevated exacerbation rate or high cigarette consumption. There is a lack of longitudinal data on the relationship between the exacerbation rate and worsening of SGRQ during disease. The aim of this study was to provide not only cross-sectional data but also information about the deterioration in quality of life over a follow-up period up to 7 years (median follow-up period of 3.33 years). Methods: We investigated questionnaire-based data of the German AATD registry concerning the relationship between SGRQ and exacerbation frequency, smoking history, forced expiratory volume in 1 second (FEV1 ) and carbon monoxide diffusion capacity (DLCO) first in crosssectional analysis and later in longitudinal analysis. Results: Eight hundred sixty-eight individuals with protease inhibitor ZZ (PiZZ) genotype with an average age of 52.6±12.8 years had an SGRQ score of 45.7±20.6. SGRQ significantly correlated with the exacerbation frequency within the last 2 years (r=0.464; P,0.001), smoking history (r=0.233; P,0.001), FEV1 (r=−0.436; P,0.001), DLCO (r=−0.333; P,0.001), and patients’ age (r=0.292; P,0.001). Individuals with occupational dust exposure had significantly worse quality of life (P,0.001). Mean annual deterioration of SGRQ in all patients with available follow-up data (n=286) was 1.21±4.45 points per year. Univariate and multivariate analysis showed a significant relationship between worsening of SGRQ/year and exacerbation frequency in the follow-up period (r=0.144; P=0.015). Conclusion: Worsening of SGRQ is associated with the exacerbation frequency in individuals with PiZZ AATD.
DOI of the first publication: 10.2147/COPD.S130925
URL of the first publication:
Link to this record: urn:nbn:de:bsz:291--ds-374611
ISSN: 1178-2005
Date of registration: 30-Sep-2022
Faculty: M - Medizinische Fakultät
Department: M - Innere Medizin
M - Medizinische Biometrie, Epidemiologie und medizinische Informatik
Professorship: M - Prof. Dr. Robert Bals
M - Prof. Dr. Stefan Wagenpfeil
Collections:SciDok - Der Wissenschaftsserver der Universität des Saarlandes

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