Clinical and Functional Outcomes Associated with Quality of Life in Patients with Lymphangioleiomyomatosis: A Cross-Sectional Study.

IF 4.6 2区 医学 Q1 RESPIRATORY SYSTEM Lung Pub Date : 2024-12-01 Epub Date: 2024-10-14 DOI:10.1007/s00408-024-00751-w
Douglas Silva Queiroz, Cibele Cristine Berto Marques da Silva, Martina Rodrigues Oliveira, Alexandre Franco Amaral, Carlos Roberto Ribeiro Carvalho, João Marcos Salge, Bruno Guedes Baldi, Celso R F Carvalho
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Abstract

Background: Lymphangioleiomyomatosis (LAM) is a rare (twenty-one per million female inhabitants) neoplastic cystic lung disease that impairs health-related quality of life (HRQoL). However, the factors associated with impaired quality of life in patients with LAM are poorly understood.

Objective: To assess the clinical, psychosocial, and functional characteristics associated with impaired quality of life in patients with LAM.

Methods: This was a cross-sectional study performed on two nonconsecutive days. HRQoL (SF-36 and CRQ), lung function tests, anxiety and depression symptoms (HADS), maximal (CPET and ISWT), and submaximal exercise capacity (6MWT) were assessed. Linear associations among outcomes were assessed using Pearson's correlation and multivariate tests.

Results: Forty-five women with LAM (46 ± 10.years; FEV1,74%pred) were evaluated. The lowest SF-36 scores were observed for general health and vitality and the highest for the physical and social domains. The lowest CRQ scores were observed for dyspnea and fatigue, and the highest were for the emotional function and self-control domains. Sixteen (35%) women had anxiety, and 8 (17%) had depression symptoms. Most of the SF-36 and CRQ domains were associated with anxiety and depression symptoms (from r = 0.4 to r = 0.7; p < 0.05) and exercise capacity (from r = 0.3 to r = 0.5; p < 0.05). Lung function parameters were weakly or not associated with quality of life domains. After multiple linear regression, HRQoL was independently associated with depression symptoms and physical capacity but not with lung function.

Conclusion: Our results show that aerobic capacity and depression symptoms are the main factors, rather than lung function, related to quality of life in patients with LAM.

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与淋巴管瘤病患者生活质量相关的临床和功能结果:一项横断面研究
背景:淋巴管瘤病(LAM)是一种罕见的(每百万女性居民中有21人患此病)肿瘤性囊肺疾病,会损害与健康相关的生活质量(HRQoL)。然而,人们对 LAM 患者生活质量受损的相关因素知之甚少:评估与 LAM 患者生活质量受损相关的临床、社会心理和功能特征:这是一项在两个非连续日进行的横断面研究。对患者的 HRQoL(SF-36 和 CRQ)、肺功能测试、焦虑和抑郁症状(HADS)、最大运动能力(CPET 和 ISWT)和亚最大运动能力(6MWT)进行了评估。使用皮尔逊相关性检验和多变量检验评估了结果之间的线性关系:对 45 名女性 LAM 患者(46 ± 10.岁;FEV1,74%pred)进行了评估。一般健康和活力的 SF-36 得分最低,身体和社交领域的 SF-36 得分最高。CRQ得分最低的是呼吸困难和疲劳,最高的是情绪功能和自我控制。16名妇女(35%)有焦虑症状,8名妇女(17%)有抑郁症状。SF-36 和 CRQ 的大多数领域都与焦虑和抑郁症状相关(从 r = 0.4 到 r = 0.7;p):我们的研究结果表明,与肺功能相比,有氧能力和抑郁症状是影响肺结核患者生活质量的主要因素。
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来源期刊
Lung
Lung 医学-呼吸系统
CiteScore
9.10
自引率
10.00%
发文量
95
审稿时长
6-12 weeks
期刊介绍: Lung publishes original articles, reviews and editorials on all aspects of the healthy and diseased lungs, of the airways, and of breathing. Epidemiological, clinical, pathophysiological, biochemical, and pharmacological studies fall within the scope of the journal. Case reports, short communications and technical notes can be accepted if they are of particular interest.
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