Exacerbations and duration of smoking abstinence are associated with the annual loss of FEV1 in individuals with PiZZ alpha-1-antitrypsin deficiency

Fähndrich, Sebastian; Bernhard, Nikolas; Lepper, Philipp M.; Vogelmeier, Claus; Seibert, Martina; Wagenpfeil, Stefan; Bals, Robert

doi:10.1016/j.rmed.2017.05.011

Cited by 12 publications

(7 citation statements)

References 28 publications

(25 reference statements)

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“…We found that frequent exacerbations were associated with accelerated loss of FEV 1 . Our findings are in accordance with previous studies of usual COPD and in AATD patients 9,10,12,13,20…”

Section: Discussionsupporting

confidence: 94%

Decline in FEV1 and hospitalized exacerbations in individuals with severe alpha-1 antitrypsin deficiency

Hiller

Piitulainen

Jehpsson

et al. 2019

COPD

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Background and aim: The value of the forced expiratory volume in one second (FEV 1 ) is useful in the diagnosis and prognosis of chronic obstructive pulmonary disease (COPD). Previous studies on lung function in individuals with severe alpha-1 antitrypsin deficiency (AATD) have shown a variable annual decline in FEV 1 (∆FEV 1 ). The aim of this study was to analyze ∆FEV 1 and to identify risk factors for ∆FEV 1 in individuals with severe AATD. Material and methods: Data on smoking habits, symptoms, results of lung function tests and exacerbations were obtained from the Swedish AATD Register and the Swedish National Patient Register (SNPR). The ∆FEV 1 was analyzed by random-effects modeling and adjusted for age and FEV 1 at baseline. Results: One hundred and four (9%) current smokers, 539 (48%) ex-smokers and 489 (43%) never-smokers were included in the study and followed-up from 1991 to 2016. A total of 584 (52%) individuals with severe AATD had COPD at inclusion. The median (IQR) annual severe exacerbation rate was 0.66 (1.4). The adjusted mean ∆FEV 1 was significantly higher in the current smokers compared with the ex-smokers and never-smokers (70 [95% CI 56–83] vs 42 [95% CI 36–48] and 32 [95% CI 25–38) mL·yr −1 ], in the middle–aged individuals compared with the young individuals (48 [95% CI 41–55] vs 32 [95% CI 18–45] mL·yr −1 ), in the individuals with respiratory symptoms at inclusion compared with the asymptomatic individuals (46 [95% CI 40–52] vs 30 [95% CI 22–38]mL·yr −1 ), and in the individuals with frequent exacerbations compared with those with infrequent exacerbations (57 [95% CI 47–68] vs 27 [95% CI 17–37] mL·yr −1 ). Conclusion: Active smoking, age, respiratory symptoms at baseline and repeated severe exacerbations of COPD are factors associated with an accelerated decline of lung function in individuals with severe AATD.

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“…We found that frequent exacerbations were associated with accelerated loss of FEV 1 . Our findings are in accordance with previous studies of usual COPD and in AATD patients 9,10,12,13,20…”

Section: Discussionsupporting

confidence: 94%

Decline in FEV1 and hospitalized exacerbations in individuals with severe alpha-1 antitrypsin deficiency

Hiller

Piitulainen

Jehpsson

et al. 2019

COPD

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“…Our review suggests that patients with AATD who have frequent exacerbations (⩾3 per year) or chronic sputum expectoration have a poorer QoL than patients without [74], and that SGRQ scores may be significantly correlated with both exacerbations and dyspnoea [76]. Exacerbations are often associated with long-term sequelae including significant, permanent loss of lung function [26,129,130]. However, exacerbations are random events that are driven by infections and outcomes can vary substantially.…”

Section: Discussionmentioning

confidence: 99%

Disease burden associated with alpha-1 antitrypsin deficiency: systematic and structured literature reviews

Miravitlles

Herepath²,

Priyendu

et al. 2022

Eur Respir Rev

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Alpha-1 antitrypsin deficiency (AATD) is a rare genetic disorder characterised by reduced levels of circulating alpha-1 antitrypsin and an increased risk of lung and liver disease. Recent reviews of AATD have focused on diagnosis, epidemiology and clinical management; comprehensive reviews examining disease burden are lacking. Therefore, we conducted literature reviews to investigate the AATD disease burden for patients, caregivers and healthcare systems. Embase, PubMed and Cochrane libraries were searched for AATD publications from database inception to June 2021, in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Most published AATD studies were small and short in duration, with variations in populations, designs, measures and outcomes, complicating cross-study comparisons. AATD was associated with significant pulmonary and hepatic morbidity. COPD, emphysema and bronchiectasis were common lung morbidities, where smoking was a key risk factor. Fibrosis and steatosis were the most common liver complications reported in patients with a PiZ allele. Health status analyses suggested a poorer quality of life for AATD patients diagnosed with COPD versus those with non-AATD-associated COPD. The burden for caregivers included loss of personal time due to caring responsibilities, stress and anxiety. AATD was also associated with high direct medical costs and healthcare resource utilisation.

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“…The results of our study also support the findings of previous limited analyses of ZZ-AATD patients. The national German registry analysis with patients who had not received augmentation therapy (n=15) and patients who did receive therapy (n=85) over a mean follow-up of 4.89 years [ 22 ] showed no difference in FEV 1 decline between groups. Neither did the analysis of the Spanish national database of patients who did not receive augmentation therapy (n=45) and patients who did receive augmentation therapy (n=77) over an average of >8 years [ 23 ].…”

Section: Discussionmentioning

confidence: 99%

“…Also, the registry does not include data about the use and timing of long-acting bronchodilators on the day of the spirometry. Fourth, several other factors are known to influence FEV 1 decline in AATD: baseline FEV 1 , smoking history, age, body mass index (BMI) and exacerbation rate [ 22 , 26 ]. In our database, no exacerbation rate or respiratory symptoms are registered, apart from the St. George's Respiratory Questionnaire score at baseline.…”

Section: Discussionmentioning

confidence: 99%

Long-term effect of α₁-antitrypsin augmentation therapy on the decline of FEV₁in deficient patients: an analysis of the AIR database

et al. 2021

Self Cite

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BackgroundPatients with ZZ (Glu342Lys) alpha-1-antitrypsin deficiency (ZZ-AATD) who received augmentation therapy with alpha-1-antitrypsin (AAT) in randomised controlled trials over 2–3 years, failed to show a significant reduction of the annual decline of FEV1.MethodsTo compare the trajectory of FEV1 change during 4 or more years in ZZ-AATD patients with emphysema receiving or not receiving intravenous augmentation therapy, a retrospective analysis of FEV1 values entered in the Alpha-1 International Registry (AIR) of ZZ-AATD patients from five different European countries: Germany, UK, Spain, Italy and The Netherlands was performed. The post-bronchodilator FEV1%predicted values for baseline and follow-up over time from patients were analysed using linear mixed effects models.ResultsData of 374 patients were analysed: 246 untreated and 128 treated with intravenous AAT augmentation therapy. The mean follow-up duration of the untreated group was 8.60 (sd±3.34) years and 8.59 (±2.62) years for the treated group. The mixed effects model analysis showed a mean FEV1 decline of −0.931% predicted per year (95% confidence interval −1.144 to −0.718) in the untreated group and a decline of −1.016% predicted per year (−1.319 to −0.7145) in the treated group. The likelihood ratio test showed no difference between the two groups (p=0.71).ConclusionIn our study population, we could not detect a significant difference in the annual decline of FEV1 by AAT augmentation treatment over an average period of 8.6 years. Other approaches are needed to validate any benefit of augmentation therapy.

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Exacerbations and duration of smoking abstinence are associated with the annual loss of FEV1 in individuals with PiZZ alpha-1-antitrypsin deficiency

Abstract: Annual decline in FEV is related to the exacerbation rate, occupational dust exposure and the duration of smoking abstinence.

Cited by 12 publications

References 28 publications

<p>Decline in FEV<sub>1</sub> and hospitalized exacerbations in individuals with severe alpha-1 antitrypsin deficiency</p>

<p>Decline in FEV<sub>1</sub> and hospitalized exacerbations in individuals with severe alpha-1 antitrypsin deficiency</p>

Disease burden associated with alpha-1 antitrypsin deficiency: systematic and structured literature reviews

Long-term effect of α₁-antitrypsin augmentation therapy on the decline of FEV₁in deficient patients: an analysis of the AIR database

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Exacerbations and duration of smoking abstinence are associated with the annual loss of FEV1 in individuals with PiZZ alpha-1-antitrypsin deficiency

Abstract: Annual decline in FEV is related to the exacerbation rate, occupational dust exposure and the duration of smoking abstinence.

Cited by 12 publications

References 28 publications

<p>Decline in FEV<sub>1</sub> and hospitalized exacerbations in individuals with severe alpha-1 antitrypsin deficiency</p>

<p>Decline in FEV<sub>1</sub> and hospitalized exacerbations in individuals with severe alpha-1 antitrypsin deficiency</p>

Disease burden associated with alpha-1 antitrypsin deficiency: systematic and structured literature reviews

Long-term effect of α1-antitrypsin augmentation therapy on the decline of FEV1in deficient patients: an analysis of the AIR database

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Long-term effect of α₁-antitrypsin augmentation therapy on the decline of FEV₁in deficient patients: an analysis of the AIR database