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

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

来源：Respiratory Medicine, 2017, 130: 98-101.

DOI：10.1016/j.rmed.2017.07.009

• Alpha-1 antitrypsin deficiency
• Pulmonary rehabilitation
• COPD
• Exercise training
• Creatinine
• Augmentation therapy

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" (AATD(AUG+)) or "off" (AATD(AUG-)) 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 (AATD(AUG+): +51 +/- 55 m, AATD(AUG-): +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 trainingrelated benefit in 6MWD. Augmentation therapy showed no effect on 6MWD adaptation during PR.

• 出版日期2017-9