Quality of life review: St George's Respiratory Questionnaire (SGRQ) scores in patients with alpha-1 antitrypsin deficiency (AATD)
| First author, year [ref.] | Population/ treatment | Subjects n | Time point | SGRQ scores | Main SGRQ outcomes | |||
| Total | Symptoms | Activity | Impact | |||||
| Bernhard, 2017 [79] | AATD (PiZZ): never-smokers | 223 | 36.9±21.8 | 44.5±23.8 | 45.3±26.8 | 28.7±21.5 | In contrast to never- and intensive (ex-) smokers, moderate-smoking PiSZ individuals had a significantly better SGRQ total score (p=0.020) and fewer exacerbations (p=0.037) than individuals with a PiZZ genotype. | |
| AATD (PiSZ): never-smokers | 33 | 22.5±21.6 | 35.0±23.9 | 25.2±29.6 | 16.7±19.3 | |||
| AATD (PiZZ): moderate (ex-) smokers (0<pack-years<30) | 491 | 46.9±19.8 | 55.7±22.7 | 58.3±22.6 | 37.3±21.0 | |||
| AATD (PiSZ): moderate (ex-) smokers (0<pack-years<30) | 44 | 38.9±25.6 | 43.3±28.2 | 48.8±30.4 | 29.0±24.3 | |||
| AATD (PiZZ): intensive (ex-) smokers (≥30 pack-years) | 126 | 53.2±16.5 | 62.8±19.7 | 67.5±18.1 | 41.9±18.7 | |||
| AATD (PiSZ): intensive (ex-) smokers (≥30 pack-years) | 33 | 59.8±19.0 | 62.1±20.7 | 71.6±20.1 | 49.2±22.3 | |||
| Piitulainen, 2017 [80] | AATD (PiZZ): never-smokers | 152 | 3.7 (0–56.3) | 2.5 (0–78.4) | 6.0 (0–59.5) | 0 (0–47.6) | PiZZ current smokers had a significantly higher median SGRQ activity score than the PiZZ never-smokers (p=0.032).# PiMM current smokers had significantly higher SGRQ activity (p<0.001), symptom (p<0.001) and total (p=0.001) scores than PiMM never-smokers. | |
| AATD (PiZZ): former smokers | 40 | 5.0 (0–34.4) | 7.5 (0–52.2) | 8.8 (0–41.1) | 0 (0–24.2) | |||
| AATD (PiZZ): current smokers | 19 | 14.2 (2.9–20.1) | 18.4 (5.9–36.3) | 24.6 (6.0–47.7)# | 2.8 (0–8.7) | |||
| AATD (PiSZ): never-smokers | 152 | 6.2 (61.8) | 11.8 (0–58.9) | 12.2 (0–66.9) | 0 (0–59.9) | |||
| AATD (PiSZ): former smokers | 40 | 4.7 (2.0–7.3) | 5.5 (0–18.4) | 12.2 (6.0–18.5) | 0 (0–0) | |||
| AATD (PiSZ): current smokers | 19 | 13.9 | 37.8 | 18.5 | 14.2 | |||
| Luisetti, 2015 [81] | AATD | 52 | Baseline | 29.8±26.3 | Patients who received AAT therapy had poorer baseline QoL versus patients who did not receive AAT therapy (p=0.001). | |||
| AATD index cases | 35 | Baseline | 41.2±24.4 | |||||
| AATD non-index cases | 17 | Baseline | 6.3±8.3 | |||||
| AATD + AAT therapy | 18 | Baseline | 52.7±20.6 | |||||
| AATD without AAT therapy | 19 | Baseline | 28.0±21.8 | |||||
| Gauvain, 2015 [76] | AATD | 273 | Baseline | 49.0±20.0 | 52.5±22.0 | 63.6±22.3 | 39.4±22.2 | The number of exacerbations in the past year was significantly associated with SGRQ score (R=0.36; p<0.0001) and SGRQ scores had the strongest association with dyspnoea (R=0.65; p<0.0001). Multivariate analysis suggested that 57% of the variability seen in SGRQ scores resulted from dyspnoea (p<0.0001), DLCO (% predicted) (p<0.001), chronic bronchitis (p=0.002), age (p=0.0088) and 6-min walk distance (p=0.037). |
| AATD: females | 101 | Baseline | 52.7±20.7 | |||||
| AATD: males | 172 | Baseline | 46.8±18.2 | |||||
| Bradi, 2015 [82] | AATD + AAT therapy | 24 | 1 year | 50±14 | AAT therapy status was significantly correlated with SGRQ scores when controlling for baseline FEV1 (p=0.014). | |||
| AATD without AAT therapy | 34±22 | |||||||
| Stolk, 2003 [14] | AATD | 22 | Baseline | 32.4±20.1 | Changes in lung density as measured by CT scan (15th percentile point and relative area <–950 HU) were correlated with SGRQ total scores (R= –0.56, p=0.007 and R=0.6, p=0.003, respectively). | |||
| AATD | 22 | 30 months | CFB: 6.5 (–2.9–17.5) | |||||
| Annunziata, 2021 [73] | AATD | 16 | Baseline | 18.0±3.0 | All the questionnaires completed at 3 months showed an increase in score compared with the questionnaire completed during the last hospital administration session (p<0.01). | |||
| AATD | 16 | 3 months | 22.6±3.3 | |||||
| Schramm, 2020 [83] | AATD (PiZZ) | 84 | 12.0 | 14.0 | 18.8 | 7.3 | There was no significant difference in SGRQ score between PiZZ ever-smokers and never-smokers, but PiZZ ever-smokers had significantly higher scores in all categories compared with never-smoking controls (symptom p=0.04, activity p=0.01, impact p=0.03, total p<0.01). | |
| Never-smoking control | 72 | 3.8 | 4.5 | 7.8 | 1.0 | |||
| Sandhaus, 2020 [84] | AATD (PiZZ or worse) + AAT therapy | 655 | Annual worsening of SGRQ total was on average 1.3 points per year worse in control group patients versus those receiving AAT therapy (95% CI 0.41–2.19, p=0.004). | |||||
| AATD without AAT therapy | 655 | |||||||
| Crossley, 2020 [85] | AATD | 187 | 45.2 (3.3–62.1) | Median SGRQ score was 45.2 (33.3–62.1) and related to the GOLD stage (p<0.001). There were significant correlations between QoL measures and spirometry, as measured by FEV1 (% predicted), FVC (% predicted), FEV1/FVC (%) and with gas transfer coefficient (% predicted) and gas trapping as measured by RV/TLC (%) (p<0.01 all comparisons). Total SGRQ correlated significantly with CT density, although the relationship was weak (r2<0.1). | ||||
| AATD plus COPD or emphysema | ||||||||
| Hogarth, 2019 [86] | AATD + severe emphysema | 20 | Baseline | 55.2±16.0 | After 6 months, SGRQ had decreased substantially compared with baseline in patients fitted with an endobronchial valve. | |||
| 20 | 6 months | CFB: –14.3±12.9 | ||||||
| 20 | 12 months | CFB: –8.2±12.9 | ||||||
| Durkan, 2019 [87] | AATD + COPD | 30 | NR | 36.5±18.5 | 42.4±41.6 | 24.9±17.3 | For the same level of COPD impairment, PiZZ patients presented with lower SGRQ scores than PiMM patients. | |
| Stockley, 2018 [88] | AATD (without obstruction) | 84 | Baseline | 14.0 (4.8–5.5) | 30.9 (11.7–57.1) | 12.2 (0–41.6) | 5.8 (0–21.4) | Baseline SGRQ scores were correlated with baseline FEV1 in patients with AATD with or without COPD (r2=0.34, p<0.0001). Annual SGRQ decline was greater for patients with AATD diagnosed with COPD who had a rapid FEV1 decline. |
| AATD + COPD | 370 | Baseline | 48.2 (33.9–62.4) | 62.5 (46.2–78.6) | 60.4 (47.4–79.7) | 34.9 (21.3–49.9) | ||
| AATD with no FEV1 decline | 35 | Baseline | 16.2 (4.8–35.5) | 35 (11.7–57.1) | 23.3 (0–41.6) | 11.2 (0–21.4) | ||
| AATD with FEV1 decline | 22 | Baseline | 11.5 (33.9–62.4) | 30.5 (46.2–78.6) | 11.7 (47.4–79.7) | 5.3 (21.3–49.9) | ||
| AATD + COPD with no FEV1 decline | 72 | Baseline | 51.8 (35.0–63.3) | 62.4 (51.5–78.1) | 66.6 (47.7–80.9) | 35.6 (22.3–49.5) | ||
| AATD + COPD with FEV1 decline | 189 | Baseline | 45.2 (30.5–61.5) | 60.5 (42.9–74.6) | 59.5 (41.4–79.9) | 34.1 (17.0–47.2) | ||
| AATD (without obstruction) | 84 | Annual decline | 0.2 (–0.8–1.1) | 0.00 (−2.5–2.0) | 0.00 (−0.8–1.4) | 0.14 (−0.5–0.9) | ||
| AATD + COPD | 370 | Annual decline | 0.7 (–0.8–2.4) | 0.21 (−2.3–2.1) | 1.2 (−0.5–3.6) | 0.4 (−1.1–2.2) | ||
| AATD with no FEV1 decline | 35 | Annual decline | 0.04 (–0.7–0.8) | –0.2 (–3.0–1.6) | 0.05 (–1.4–1.3) | 0.03 (–0.6–0.7) | ||
| AATD with FEV1 decline | 22 | Annual decline | 0.5 (–1.0–1.9) | 0.9 (–1.5–2.3) | 0.9 (0.0–3.0) | 0.3 (–1.0–1.3) | ||
| AATD + COPD with no FEV1 decline | 72 | Annual decline | 0.5 (–0.8–1.5) | –0.2 (–1.9–1.3) | 0.7 (–0.4–2.3) | 0.1 (–0.9–1.5) | ||
| AATD + COPD with FEV1 decline | 189 | Annual decline | 1.07 (–1.1–2.9) | 0.5 (–2.6–2.7) | 1.5 (–0.5–4.3) | 0.7 (–1.0–3.0) | ||
| Karl, 2017 [75] | AATD + COPD | 131 | NR | 44.8±17.2 | No significant differences in SGRQ scores were observed between patients with AATD diagnosed with COPD who were recipients and non-recipients of AAT therapy. | |||
| AATD + COPD + AAT therapy | 106 | NR | 46.6±16.4 | |||||
| AATD + COPD without AAT therapy | 25 | NR | 37.5±20.2 | |||||
| Chapman, 2015 [12] | AATD + emphysema + A1P1 therapy | 93 | Baseline | 44.3±17.1 | 46.5±22.7 | 62.1±18.6 | 33.6±18.4 | Improvements were reported in only the SGRQ symptom domain after 24 months of treatment. |
| AATD + emphysema + placebo | 87 | Baseline | 42.4±18.0 | 44.1±24.8 | 60.1±21.4 | 31.4±17.6 | ||
| AATD + emphysema + AAT therapy | 93 | 24 months | CFB: 1.4±11.1 | CFB: –1.4±16.7 | CFB: 1.7±12.4 | CFB: 2.1±14.8 | ||
| AATD + emphysema + placebo | 87 | 24 months | CFB: 2.2±11.7 | CFB: 2.0±20.1 | CFB: 2.6±13.5 | CFB: 1.8±12.5 | ||
| Ponce, 2014 [89] | AATD + COPD | 573 | Baseline | 46.0±17.8 | Poorer SGRQ scores were observed in obese versus non-obese AATD patients diagnosed with COPD. | |||
| AATD + COPD | 573 | 5 years | 51.0±17.7 | |||||
| Holm, 2013 [90] | AATD + COPD | 578 | 48.5±19.4 | AATD patients diagnosed with COPD had an SGRQ total score almost 5 points higher than non-AATD patients diagnosed with COPD when adjusting for demographic and health characteristics. | ||||
| Lascano, 2010 [91] | AATD + COPD + AAT therapy: overweight | 241 | 1 year | 47.2±16.0 | SGRQ scores were higher in obese patients versus patients with a normal BMI; however, the obese patients had similar FEV1 to the normal BMI group, but more comorbidity. Underweight patients had worse QoL and significantly lower FEV1 versus normal BMI individuals. | |||
| AATD + COPD + AAT therapy: obese | 104 | 1 year | 48.7±17.1 | |||||
| AATD + COPD + AAT therapy: morbidly obese | 61 | 1 year | 55.5±17.1 | |||||
| AATD + COPD + AAT therapy: normal BMI | 204 | 1 year | 43.4±16.6 | |||||
| Campos, 2009 [74] | AATD + COPD + AAT therapy | 922 | Baseline | 48.1±18.4 | Subjects with frequent exacerbations had the worst baseline HRQoL scores, as well as more physician visits, emergency room visits and hospitalisations. | |||
| AATD + COPD + AAT therapy; no exacerbations | 83 | Baseline | 37.3±17.3 | 33.1±43.5 | 57.5±23.4 | 27.0±16.5 | ||
| AATD + COPD + AAT therapy; 1–2 exacerbations per year | 391 | Baseline | 44.5±16.6 | 43.0±22.1 | 64.1±21.2 | 33.6±16.5 | ||
| AATD + COPD + AAT therapy; ≥3 exacerbations per year | 448 | Baseline | 52.4±16.5 | 54.0±21.4 | 71.0±20.5 | 41.2±17.6 | ||
| Dawkins, 2009 [92] | AATD + COPD with fast FEV1 decline | 33 | 49.6±20.1 | SGRQ total scores in fast decliners as measured by FEV1 were not significantly different from middle or slow decliners. | ||||
| AATD + COPD with middle FEV1 decline | 34 | 56.2±18.5 | ||||||
| AATD + COPD with slow FEV1 decline | 34 | 51.6±24.7 | ||||||
| AATD with other comorbidity | ||||||||
| Stone, 2016 [93] | AATD + lung transplant | 32 | Baseline | 64.2±2.5 | 75.4±2.5 | 93 (73–95) | 50.1±2.9 | Pre-transplant, although matched for FEV1, the transplant group had worse health status. Post-transplant, physiology and health status improved significantly (p<0.002). |
| AATD + no transplant | 48 | Baseline | 55.3±2.0 | 67.4±2.2 | 79 (59–91) | 40.3±2.4 | ||
| AATD + pre-transplant | 14 | Baseline | 67.5 (51.0–77.8) | 76.5 (64.5–88.5) | 93.0 (4.8–98.3) | 50.0 (31.3–65.5) | ||
| AATD + post-transplant | 14 | 1 year | 7.5 (5.0–13.8) | 14.0 (9.0–30.3) | 11.0 (1.3–20.3) | 4.5 (1.0–9.5) | ||
| Dowson, 2002 [78] | AATD with chronic sputum expectoration | 50 | 64.4 (48.3–74.4) | 75.6 (68.0–83.7) | 82.9 (60.4–100) | 49.9 (33.0–62.7) | Patients with chronic sputum expectoration had worse health status, as assessed by SGRQ (p<0.01 for all domains), than patients who did not. | |
| AATD without chronic sputum expectoration | 67 | 42.0 (23.9–59.5) | 47.6 (28.9–66.7) | 59.5 (32.7–86.3) | 28.9 (11.7–47.6) | |||
Data are presented as mean±sd or median (range), unless otherwise stated. PiSZ/ZZ: Pi (or SERPINA1 gene) SZ and ZZ alleles; AAT: alpha-1 antitrypsin; QoL: quality of life; DLCO: diffusing capacity of the lung for carbon monoxide; FEV1: forced expiratory volume in 1 s; CFB: change from baseline; CT: computed tomography; GOLD: Global Initiative for Chronic Obstructive Lung Disease; FVC: forced vital capacity; RV: residual volume; TLC: total lung capacity; NR: not reported; A1P1: α1 proteinase inhibitor; BMI: body mass index; HRQoL: health-related QoL. #: p=0.032 versus PiZZ never-smokers.