Comparison of exercise training responses in COPD patients with and without Alpha-1 antitrypsin deficiency

Jarosch, Inga; Hitzl, Wolfgang; Koczulla, Andreas Rembert; Wencker, Marion; Welte, Tobias; Gloeckl, Rainer; Janciauskiene, Sabina; Kenn, Klaus

doi:10.1016/j.rmed.2017.07.009

Cited by 15 publications

(13 citation statements)

References 14 publications

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“…Three studies were RCT, [60][61][62] one study was an open-label extension of an RCT 46 and four studies were retrospective cohort studies. [66][67][68][69] The evidence profiles for each outcome can be found in Appendix S2 (Supplementary Information).…”

Section: Resultsmentioning

confidence: 99%

“…There was no significant difference between AAT augmentation therapy group and control group in functional capacity change, measured as change in an incremental shuttle walk test in an RCT 60 and as change in 6-min walking distance (6MWD) in an observational study. 67 Mortality. None of the reviewed studies were powered to evaluate mortality as an outcome.…”

Section: Exacerbations/lung Infections/hospitalizationsmentioning

confidence: 99%

“…103 The benefit from the exercise component of pulmonary rehabilitation appears to be similar in AATD-related and non-AATDrelated COPD. 67 Education and self-management support, including COPD action plans, 104 are useful components of chronic disease management for COPD.…”

Section: Non-pharmacological Managementmentioning

confidence: 99%

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Diagnosis and treatment of lung disease associated with alpha one‐antitrypsin deficiency: A position statement from the Thoracic Society of Australia and New Zealand*

et al. 2020

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AATD is a common inherited disorder associated with an increased risk of developing pulmonary emphysema and liver disease. Many people with AATD‐associated pulmonary emphysema remain undiagnosed and therefore without access to care and counselling specific to the disease. AAT augmentation therapy is available and consists of i.v. infusions of exogenous AAT protein harvested from pooled blood products. Its clinical efficacy has been the subject of some debate and the use of AAT augmentation therapy was recently permitted by regulators in Australia and New Zealand, although treatment is not presently subsidized by the government in either country. The purpose of this position statement is to review the evidence for diagnosis and treatment of AATD‐related lung disease with reference to the Australian and New Zealand population. The clinical efficacy and adverse events of AAT augmentation therapy were evaluated by a systematic review, and the GRADE process was employed to move from evidence to recommendation. Other sections address the wide range of issues to be considered in the care of the individual with AATD‐related lung disease: when and how to test for AATD, changing diagnostic techniques, monitoring of progression, disease in heterozygous AATD and pharmacological and non‐pharmacological therapy including surgical options for severe disease. Consideration is also given to broader issues in AATD that respiratory healthcare staff may encounter: genetic counselling, patient support groups, monitoring for liver disease and the need to establish national registries for people with AATD in Australia and New Zealand.

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Section: Resultsmentioning

confidence: 99%

Section: Exacerbations/lung Infections/hospitalizationsmentioning

confidence: 99%

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Diagnosis and treatment of lung disease associated with alpha one‐antitrypsin deficiency: A position statement from the Thoracic Society of Australia and New Zealand*

et al. 2020

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“…A study comparing the effects of pulmonary rehabilitation in patients with AATD-related COPD and general COPD concluded that the benefits in terms of physical performance increase were similar in both groups of patients. 32 However, data on emphysema distribution, comorbidities, and individual pharmacological therapy were not available in the database used for this study and so it cannot be excluded that patient comorbidities, which can have profound effects on quality of life, may have influenced results. The effects of exercise on specific clinical outcomes in AATD are likely positive but require further investigation.…”

Section: Non-pharmacological Interventions In the Management Of Aatd-associated Lung Diseasementioning

confidence: 99%

Management of lung disease in alpha-1 antitrypsin deficiency: what we do and what we do not know

Campos

2021

Therapeutic Advances in Chronic Disease

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Management of lung disease in patients with alpha-1 antitrypsin deficiency (AATD) includes both non-pharmacological and pharmacological approaches. Lifestyle changes with avoidance of environmental pollutants, including tobacco smoke, improving exercise levels and nutritional status, all encompassed under a disease management program, are crucial pillars of AATD management. Non-pharmacological therapies follow conventional treatment guidelines for chronic obstructive pulmonary disease. Specific pharmacological treatment consists of administering exogenous alpha-1 antitrypsin (AAT) protein intravenously (augmentation therapy). This intervention raises AAT levels in serum and lung epithelial lining fluid, increases anti-elastase capacity, and decreases several inflammatory mediators in the lung. Radiologically, augmentation therapy reduces lung density loss over time, thus delaying disease progression. The effect of augmentation therapy on other lung-related outcomes, such as exacerbation frequency/length, quality of life, lung function decline, and mortality, are less clear and questions regarding dose optimization or route of administration are still debatable. This review discusses the rationale and available evidence for these interventions in AATD.

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“…Dadurch hat sie im frühen Stadium der Erkrankung das Potenzial, den Krankheitsverlauf positiv zu beeinflussen [1,7]. Dies gilt auch für Patienten mit einem Alpha-1-Antitrypsin-Mangel [8].…”

Section: Indikationen Und Evidenzunclassified

Rehabilitation in der Pneumologie

et al. 2021

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Comparison of exercise training responses in COPD patients with and without Alpha-1 antitrypsin deficiency

Abstract: Independently of the genetic variant of AAT, COPD patients achieved the same training-related benefit in 6MWD. Augmentation therapy showed no effect on 6MWD adaptation during PR.

Cited by 15 publications

References 14 publications

Diagnosis and treatment of lung disease associated with alpha one‐antitrypsin deficiency: A position statement from the Thoracic Society of Australia and New Zealand*

Diagnosis and treatment of lung disease associated with alpha one‐antitrypsin deficiency: A position statement from the Thoracic Society of Australia and New Zealand*

Management of lung disease in alpha-1 antitrypsin deficiency: what we do and what we do not know

Rehabilitation in der Pneumologie

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