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特发性肺纤维化患者短期应用吡非尼酮治疗的疗效观察

Short-Term Effects of a Multimodal 3-Week Inpatient Pulmonary Rehabilitation Programme for Patients with Sarcoidosis: The ProKaSaRe Study.

机构信息

Centre for Public Health and Healthcare, Hannover Medical School, Hannover, Germany.

Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research (DZL), Hannover, Germany.

出版信息

Respiration. 2018;95(5):343-353. doi: 10.1159/000486964. Epub 2018 Feb 27.

Abstract

BACKGROUND

Real-world data on the effects of a multicomponent pulmonary rehabilitation (PR) for patients with sarcoidosis are scarce.

OBJECTIVE

To describe characteristics of patients with sarcoidosis referred for a 3-week inpatient PR, to assess the effects of PR on their quality of life (QoL) and clinical outcomes, and to investigate whether there are specific subgroups who particularly benefit from PR.

METHODS

Using a prospective multicentre study design, data regarding 6-min walking distance (6MWD), QoL (Saint George's Respiratory Questionnaire, SGRQ), and the secondary outcomes of dyspnoea and psychological burden (fatigue, anxiety, and depression) were collected.

RESULTS

We included 296 patients in the study (average age 49.1 ± 9.7 years, 47% female, average vital capacity 3.5 ± 1.0 L [87.0 ± 20.6 predicted]). The 6MWD improved by the end of the rehabilitation by 39.8 m on average (p < 0.0001; standardised response mean, SRM = 0.61), SGRQ showed significant improvements in all 3 domains, and the total score (p < 0.001) improved by 5.69-8.28 points (SRM 0.46-0.62). For the secondary outcomes, significant improvement (p < 0.001) was seen for all measured parameters, e.g., dyspnoea (modified Medical Research Council Scale, mMRC), fatigue (Fatigue Assessment Scale [FAS]; SRM = -0.71), anxiety and depression (Hospital Anxiety and Depression Scale [HADS]; SRM -0.58/-0.38), and generic QoL (measured by the SF-36 scales of physical and mental health; SRM 0.31/0.55).

CONCLUSIONS

Our results provide the first documented evidence that PR is a promising complementary therapy option for sarcoidosis patients who remain subjectively symptomatic despite optimised outpatient medical treatment.

摘要

背景

关于结节病患者多组分肺康复(PR)效果的真实世界数据很少。

目的

描述因进行为期 3 周住院 PR 而被转诊的结节病患者的特征,评估 PR 对其生活质量(QoL)和临床结局的影响,并探讨是否存在特别受益于 PR 的特定亚组。

方法

采用前瞻性多中心研究设计,收集 6 分钟步行距离(6MWD)、QoL(圣乔治呼吸问卷,SGRQ)以及呼吸困难和心理负担(疲劳、焦虑和抑郁)等次要结局的数据。

结果

本研究纳入了 296 例患者(平均年龄 49.1±9.7 岁,47%为女性,平均肺活量 3.5±1.0 L[87.0±20.6 预计值])。康复结束时,6MWD 平均提高了 39.8 m(p<0.0001;标准化反应均值,SRM=0.61),SGRQ 在所有 3 个领域均显示出显著改善,总分(p<0.001)提高了 5.69-8.28 分(SRM 0.46-0.62)。对于次要结局,所有测量参数均显示出显著改善(p<0.001),例如呼吸困难(改良医学研究委员会量表,mMRC)、疲劳(疲劳评估量表[FAS];SRM=-0.71)、焦虑和抑郁(医院焦虑和抑郁量表[HADS];SRM=-0.58/-0.38)和一般 QoL(通过 SF-36 量表的身体和心理健康衡量);SRM 0.31/0.55)。

结论

我们的研究结果首次提供了有说服力的证据,表明 PR 是一种有前途的补充治疗选择,适用于尽管接受了优化的门诊药物治疗但仍有主观症状的结节病患者。

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