Department of Physical Therapy, School of Medicine, University of Sao Paulo, São Paulo, SP, Brazil.
Divisão de Pneumologia, Faculdade de Medicina, Instituto Do Coração, Hospital das Clínicas HCFMUSP, Universidade de São Paulo, São Paulo, SP, Brazil.
Lung. 2024 Dec;202(6):757-765. doi: 10.1007/s00408-024-00751-w. Epub 2024 Oct 14.
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.
To assess the clinical, psychosocial, and functional characteristics associated with impaired quality of life in patients with LAM.
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.
Forty-five women with LAM (46 ± 10.years; FEV74%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.
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.
淋巴管平滑肌瘤病（LAM）是一种罕见的（每百万女性居民中有 21 例）肿瘤性囊性肺部疾病，会损害健康相关生活质量（HRQoL）。然而，患有 LAM 的患者生活质量受损的相关因素尚未得到充分理解。
评估与 LAM 患者生活质量受损相关的临床、心理社会和功能特征。
这是一项在两天内进行的横断面研究。评估了 HRQoL（SF-36 和 CRQ）、肺功能测试、焦虑和抑郁症状（HADS）、最大（CPET 和 ISWT）和亚最大运动能力（6MWT）。使用 Pearson 相关和多变量检验评估结果之间的线性关联。
共评估了 45 名患有 LAM 的女性（46±10 岁；FEV74%pred）。SF-36 的最低得分出现在一般健康和活力方面，而最高得分出现在身体和社会领域。CRQ 的最低得分出现在呼吸困难和疲劳方面，而最高得分出现在情感功能和自我控制领域。16 名（35%）女性有焦虑症状，8 名（17%）有抑郁症状。大多数 SF-36 和 CRQ 领域与焦虑和抑郁症状（从 r=0.4 到 r=0.7；p<0.05）和运动能力（从 r=0.3 到 r=0.5；p<0.05）相关。肺功能参数与生活质量领域的相关性较弱或无相关性。经过多元线性回归分析，HRQoL 与抑郁症状和体力独立相关，但与肺功能无关。
我们的结果表明，有氧运动能力和抑郁症状是与 LAM 患者生活质量相关的主要因素，而不是肺功能。
