Comparison of exercise training responses in COPD patients with and without Alpha-1 antitrypsin deficiency. (September 2017)

• Record Type: Journal Article
• Authors: Jarosch, Inga; Hitzl, Wolfgang; Koczulla, Andreas Rembert; Wencker, Marion; Welte, Tobias; Gloeckl, Rainer; Janciauskiene, Sabina; Kenn, Klaus
• Is Part Of: Respiratory medicine. Volume 130(2017)
• Page Start: 98
• Page End: 101
• Publication Date: 2017-09
• Subjects: Alpha-1 antitrypsin deficiency -- Pulmonary rehabilitation -- COPD -- Exercise training -- Creatinine -- Augmentation therapy; 6MWD 6-min walk distance -- 6MWT 6-min walk test -- AAT Alpha-1 antitrypsin -- AATD alpha-1 antitrypsin deficiency -- AATDAUG+ Alpha-1 antitrypsin deficiency patients with alpha-1 antitrypsin augmentation therapy -- AATDAUG- Alpha-1 antitrypsin deficiency patients without alpha-1 antitrypsin augmentation therapy -- ATS American Thoracic Society -- COPD chronic obstructive pulmonary disease -- CPET Cardiopulmonary Exercise Test -- PiZZ Protease Inhibitor "ZZ" -- PR Pulmonary Rehabilitation -- SF36 Short Form 36 quality of life questionnaire; Chest -- Diseases -- Periodicals; Chest -- Diseases -- Great Britain -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Appareil respiratoire -- Maladies -- Périodiques; Thorax -- Maladies -- Périodiques; Appareil respiratoire -- Maladies -- Traitement -- Périodiques; Electronic journals; 616.2
• DOI: 10.1016/j.rmed.2017.07.009
• Languages: English
• ISSNs: 0954-6111

Abstract

Abstract: Background: The benefits of pulmonary rehabilitation (PR) on fatigue-resistant skeletal muscle fibre type I have been found to be smaller in COPD patients with alpha-1 antitrypsin deficiency (AATD) than in those without AATD. Alpha-1 antitrypsin (AAT) augmentation therapy was suggested as a potential factor of influence. Whether this finding mirrors different improvements in 6-min walk distance (6MWD) between both groups remains unknown. Methods: 140 patients with AATD-related COPD (phenotype PiZZ, FEV1 : 31 ± 8%pred.) and 280 COPD patients without AATD (FEV1 : 31 ± 8%pred.) were matched for baseline 6MWD and included in a retrospective analysis. AATD patients were divided into those "on" (AATDAUG+ ) or "off" (AATDAUG- ) augmentation therapy. 6MWD was assessed pre and post an inpatient 4-week PR program. Plasma level of creatinine was analysed at baseline. Results: In AATD and COPD patients with comparable initial 6MWD (331 ± 106 m and 326 ± 101 m, p = n.s.), improvements in 6MWD following PR were similar (+49 ± 49 m and +53 ± 52 m, intra-group change: p < 0.001). Notably, 68% of AATD and 65% of COPD responded well with a clinically relevant 6MWD improvement of ≥30 m. The improvement in 6MWD was independent of gender, age, pack years, SF36 mental score and body mass index. The augmentation therapy with AAT did not influence 6MWD outcome (AATDAUG+ : +51 ± 55 m, AATDAUG- : +47 ± 40 m, p = n.s.). Only in AATD group, higher baseline creatinine levels and lower 6MWD were positive predictors for the PR-related increase in 6MWD. Conclusions: 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. Highlights: Pulmonary rehabilitation is beneficial in AATD and COPD patients. Walk distance improved to comparable extent in AATD and COPD patients after PR. Small walk distance and high creatinine levels predicted a good PR response in AATD. Alpha-1 antitrypsin augmentation therapy did not influence walk distance outcome.