Department of Respiratory Medicine and Pulmonary Rehabilitation, Schoen Klinik Berchtesgadener Land, Malterhoeh 1, 83471 Schoenau am Koenigssee, Germany; Universities of Giessen and Marburg Lung Center (UGMLC), University of Marburg, German Center for Lung Research (DZL), Marburg, Germany.
Research Office (biostatistics), Paracelsus Medical University, Strubergasse 21, 5020 Salzburg, Austria.
Respir Med. 2017 Sep;130:98-101. doi: 10.1016/j.rmed.2017.07.009. Epub 2017 Jul 15.
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.
140 patients with AATD-related COPD (phenotype PiZZ, FEV: 31 ± 8%pred.) and 280 COPD patients without AATD (FEV: 31 ± 8%pred.) were matched for baseline 6MWD and included in a retrospective analysis. AATD patients were divided into those "on" (AATD) or "off" (AATD) augmentation therapy. 6MWD was assessed pre and post an inpatient 4-week PR program. Plasma level of creatinine was analysed at baseline.
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: +51 ± 55 m, AATD: +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.
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.
已经发现，与没有 AATD 的 COPD 患者相比，α-1 抗胰蛋白酶缺乏症（AATD）的 COPD 患者的肺康复（PR）对耐疲劳的骨骼肌纤维 I 型的益处较小。α-1 抗胰蛋白酶（AAT）增强疗法被认为是一个潜在的影响因素。这一发现是否反映了两组之间 6 分钟步行距离（6MWD）的不同改善尚不清楚。
140 名患有 AATD 相关 COPD（表型 PiZZ，FEV：31±8%预测值）的患者和 280 名没有 AATD 的 COPD 患者（FEV：31±8%预测值）在基线 6MWD 上进行匹配，并纳入回顾性分析。AATD 患者分为“接受”（AATD）或“未接受”（AATD）增强治疗。在住院 4 周的 PR 计划前后评估 6MWD。在基线时分析血浆肌酐水平。
在初始 6MWD 相当的 AATD 和 COPD 患者中（331±106m 和 326±101m，p=n.s.），PR 后 6MWD 的改善相似（+49±49m 和+53±52m，组内变化：p<0.001）。值得注意的是，68%的 AATD 和 65%的 COPD 患者对 6MWD 改善有良好的反应，改善幅度超过 30m，这具有临床意义。6MWD 的改善与性别、年龄、吸烟年数、SF36 精神评分和体重指数无关。AAT 的增强治疗对 6MWD 结果没有影响（AATD：+51±55m，AATD：+47±40m，p=n.s.）。只有在 AATD 组中，较高的基线肌酐水平和较低的 6MWD 是 PR 相关 6MWD 增加的正预测因子。
独立于 AAT 的遗传变异，COPD 患者在 6MWD 方面获得了相同的训练相关益处。增强治疗对 PR 期间 6MWD 的适应没有影响。
