LungenClinic Grosshansdorf, Airway Research Center North, Member of the German Center for Lung Research, Grosshansdorf, Germany.
Pulmonary Research Institute at LungenClinic Grosshansdorf, Airway Research Center North, Member of the German Center for Lung Research, Grosshansdorf, Germany.
Thorax. 2016 Jul;71(7):662-3. doi: 10.1136/thoraxjnl-2015-207852. Epub 2016 Mar 11.
Sporadic lymphangioleiomyomatosis (LAM) is an orphan lung disease for which daily physical activity has not been studied so far and it is unclear whether a disease-specific impact beyond airflow limitation exists. Clinical correlates indicating reduced physical activity in addition to established parameters like airflow limitation and hypoxaemia are largely undetermined.
We measured physical activity (steps per day, SPD; physical activity level, PAL; minutes of moderate activity, MMA) in 34 women with LAM, 32 FEV1-matched female patients with COPD and 15 age-matched healthy women for 1 week using an accelerometer. In addition, we assessed lung function measurements, questionnaires for generic and respiratory health status (12-Item Short Form Survey, SF-12; St. George's Respiratory Questionnaire, SGRQ), dyspnoea (modified Medical Research Council dyspnoea scale, mMRC) and fatigue (Multidimensional Fatigue Inventory, MFI-20).
Patients with LAM (mean age 52.7 years, mean FEV1 62.7% predicted) showed reduced SPD, PAL and MMA (p<0.01) compared with healthy controls and reduced MMA (p=0.032) compared with female patients with COPD (mean age 65.2 years, mean FEV1 62.6% predicted). In multivariate regression analyses, adjusting for FEV1 and long-term oxygen therapy, either generic health status (SF-12 physical health) or fatigue (MFI-20) were the strongest independent predictors for SPD in patients with LAM (p=0.006 and p=0.004, respectively).
Physical activity in daily life is substatially reduced in LAM, when compared with healthy controls and COPD - indicating a disease specific impact. The regular assessment of fatigue and generic health status may improve disease management in LAM by taking daily physical activity of patients with LAM more adequately into account.
散发性淋巴管平滑肌瘤病（LAM）是一种罕见的肺部疾病，目前尚未研究其日常体力活动，也不清楚是否存在超越气流受限的疾病特异性影响。除了气流受限和低氧血症等既定参数外，表明体力活动减少的临床相关性在很大程度上尚未确定。
我们使用加速度计在 34 名 LAM 女性患者、32 名 FEV1 匹配的 COPD 女性患者和 15 名年龄匹配的健康女性中测量了 1 周的日常体力活动（步数/天、PAL；中强度活动分钟数，MMA）。此外，我们评估了肺功能测量、通用和呼吸健康状况问卷（12 项简短表格调查，SF-12；圣乔治呼吸问卷，SGRQ）、呼吸困难（改良医学研究委员会呼吸困难量表，mMRC）和疲劳（多维疲劳量表，MFI-20）。
LAM 患者（平均年龄 52.7 岁，平均 FEV1 预测值 62.7%）与健康对照组相比，SPD、PAL 和 MMA 明显降低（p<0.01），与 COPD 女性患者相比，MMA 明显降低（p=0.032）（平均年龄 65.2 岁，平均 FEV1 预测值 62.6%）。在多元回归分析中，调整 FEV1 和长期氧疗后，通用健康状况（SF-12 身体健康）或疲劳（MFI-20）是 LAM 患者 SPD 的最强独立预测因子（分别为 p=0.006 和 p=0.004）。
与健康对照组和 COPD 相比，LAM 患者的日常生活体力活动明显减少，表明存在疾病特异性影响。定期评估疲劳和通用健康状况可以通过更充分地考虑 LAM 患者的日常体力活动，更好地管理 LAM 疾病。
