Usefulness of the CAT, LCOPD, EQ-5D and COPDSS Scales in Understanding the Impact of Lung Disease in Patients with Alpha-1 Antitrypsin Deficiency

Abstract

Alpha-1-antitrypsin deficiency (AATD) is an inherited disorder responsible for early onset emphysema associated with a significant impairment of health-related quality of life (HRQoL). Our aim was to assess the usefulness of different instruments to evaluate the HRQoL in patients with AATD compared to non-AATD COPD. Observational, cross-sectional study in which all patients filled out a series of questionnaires: the COPD severity score (COPDSS), the EuroQoL 5-Dimensions (EQ-5D), the Living with COPD (LCOPD) and the COPD Assessment Test (CAT).

A total of 96 patients were included, 35 with AATD (mean age 56.5 yrs, 57.1% male and mean FEV₁(%) 48.7% and 61 non-AATD COPD (70.3 yrs, 80.3% men and FEV₁(%) 47%. The questionnaire scores were similar, with a tendency towards worse scores in AATD for the EQ-5D (VAS) (64.8 (20.2) vs. 71.6 (17.1); p = 0.08). The correlations between the different scores and FEV1(%) were significant in both groups for COPDSS and LCOPD, but not for CAT and EQ-5D. In general, the correlations of scores with FEV₁(%) were stronger for AATD compared with non-AATD COPD patients: COPDSS r = −0.570, p < 0.01 for AATD and r = −0.260, p < 0.05 for COPD; LCOPD r = −0.502, p < 0.001 for AATD and r = −0.304, p < 0.05 for non-AATD COPD.

Patients with AATD have a similar degree of HRQoL impairment as older subjects with non-AATD COPD and showed a stronger correlation between HRQoL measurements and lung function impairment compared with non-AATD COPD. This may be related to the characteristics of the disease in these patients who are usually younger, with less co-morbidity and lower smoking consumption.

Keywords :

• Alpha-1-antitrypsin deficiency
• quality of life
• COPD
• CAT
• EQ-5D
• COPDSS

Acknowledgments

The authors wish to thank Albert Gabarrús (IDIBAPS. Hospital Clínic, Barcelona) for his support in the statistical analysis.

Grifols has provided support through an unrestricted grant for the development of the Catalan Center of Excellence for AATD. Sandra Manca from the Institute of Respiratory Diseases, Sassari University (Italy) has been a recipient of an Erasmus Placement fellowship.

Declaration of Interest Statement

Marc Miravitlles has received speaker fees from Boehringer Ingelheim, Pfizer, AstraZeneca, Bayer Schering, Novartis, Talecris-Grifols, Takeda-Nycomed, Merck, Sharp & Dohme and Novartis, and consulting fees from Boehringer Ingelheim, Pfizer, GSK, AstraZeneca, Bayer Schering, Novartis, Almirall, Merck, Sharp & Dohme, Talecris-Grifols and Takeda-Nycomed. The other authors have no conflict of interest to disclosure related to this article. The authors are responsible for the content and writing of this paper.