Clinical and functional outcomes associated with quality of life in patients with lymphangioleiomyomatosis: a cross-sectional study

doi:10.21203/rs.3.rs-4714749/v1

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Clinical and functional outcomes associated with quality of life in patients with lymphangioleiomyomatosis: a cross-sectional study

https://doi.org/10.21203/rs.3.rs-4714749/v1

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published 14 Oct, 2024

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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; FEV_1,74%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 dyspnoea 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.

Cystic lung disease

Exercise

Quality of life

Psychosocial aspects

Lung function

Lymphangioleiomyomatosis (LAM) is a rare neoplastic cystic lung disease (twenty-one per million female inhabitants) that mainly affects women of reproductive age¹. LAM is characterized by the abnormal proliferation of smooth muscle-like LAM cells, resulting in vascular and airway obstruction and cyst formation^1,2. The main clinical characteristics include progressive dyspnoea, pneumothorax, and chylothorax³ which limit exercise and reduce health-related quality of life (HRQoL). Although mTOR inhibitors have modified the prognosis of patients with LAM, there is no cure for LAM, and improving quality of life is an essential goal for these patients⁴. Some studies have assessed the quality of life in this population; since there is no specific questionnaire for LAM, they have primarily used the Saint George Respiratory Questionnaire (SGRQ)⁵ or the Short-Form-36 (SF-36)^6,7. Regardless of the instrument used, all studies demonstrated a significant impairment in patient's HRQoL.

Specific and generic questionnaires are used to assess the quality of life. Generic questionnaires are considered best for including general aspects of chronic disease, but their use reduces the number of items that refer to specific clinical conditions. On the other hand, disease-specific questionnaires were developed to assess symptoms and limitations, and they have the strong advantage of being able to detect small variations during the course of the disease⁸. The SF-36 is the generic HRQoL questionnaire most commonly used in clinical practice and is used to assess patients with chronic respiratory diseases (CRDs), such as chronic obstructive pulmonary disease (COPD)⁹ and idiopathic pulmonary fibrosis (IPF)¹⁰. In addition, the Chronic Respiratory Questionnaire (CRQ) is a respiratory-specific questionnaire widely used to assess the health status of patients with CRDs. Although the SGRQ is also used for patients with CRDs, the CRQ has been demonstrated to be superior since it is more responsive to interventions, such as pulmonary rehabilitation programs¹¹.

A previous study compared the generic (SF-36) and the specific (SGRQ) HRQoL questionnaires in patients with IPF¹⁰, and the authors demonstrated that the specific tool better expressed the HRQoL in this population. Another study¹² also compared the same questionnaires (SF-36 and SGRQ) in COPD patients and reported that the SGRQ had better discriminative ability than the SF-36, especially in the symptoms domain. The authors concluded that the SF-36 is not an appropriate instrument for determining the affective state of COPD patients.

There is no validated questionnaire for assessing HRQoL in LAM patients, and different instruments have been used, such as SF-36⁷, SGRQ^5,7,13, and COPD Assessment Test¹⁴. Oliveira and coworkers¹³ demonstrated that patients with tuberous sclerosis complex-LAM presented lower scores in the emotional health and vitality domains than did those with sporadic-LAM. Previous studies have also shown that LAM patients have worse scores in the physical and emotional domains^7,15. Nonetheless, the factors associated with quality of life in LAM patients have not yet been established. Therefore, the present study aimed to determine the clinical, psychosocial, and functional factors associated with impaired quality of life in LAM patients.

Participants

This cross-sectional single-center study was conducted from September 2018 to March 2021 and included a cohort of women with LAM from a tertiary university hospital. The diagnosis of LAM was based on the current guidelines^3,16. The protocol was approved by the Ethics Committee (90196617.1.0000.0068), and all included patients provided written consent. The patients were clinically stable (no exacerbation and/or pneumothorax for the last six weeks)^3,16 and had a peripheral resting oxygen saturation (SpO₂) ≥ 89% at room air. The exclusion criteria were supplemental oxygen use, other CRDs, uncontrolled heart disease, pregnancy, or any limiting condition to performing the exercise tests.

Assessments

Individuals with LAM were assessed for clinical, exercise capacity, and psychosocial outcomes. The assessments included pulmonary function tests (Spirometry and body plethysmography to quantify the lung volumes, capacities, and diffusion capacity for carbon monoxide; dyspnoea and leg fatigue perception during exercise; exercise capacity (cardiopulmonary exercise testing, incremental shuttle, and six-minute walking tests); health-related quality of life (Short form-36 (SF-36) and chronic respiratory questionnaire (CRQ); and, anxiety and depression symptoms (Hospital Anxiety and depression scale (HADS), All outcomes were assessed according to international guidelines and further details for each assessment are in the online supplement due to words limit.

Study Design

Patients were assessed during two nonconsecutive visits within one-week intervals. During the first hospital visit, clinical characteristics, quality of life, psychosocial questionnaires, and anthropometric data were obtained. The participants also performed PFTs and two 6-minute walking tests (6MWTs) with a 30-min. of recovery between tests. After the second 6MWT and 30 min of recovery, patients were randomly assigned (http://www.randomization.com) to either the cardiopulmonary exercise test (CPET) or the incremental shuttle walk test (ISWT) by an investigator not involved in the study. The remaining assessments were performed during the second visit (Fig. 1).

Statistical Analysis

The Kolmogorov–Smirnov test was used to assess data normality. Data was reported as the mean ± standard deviation (SD) or median and 25–75% interquartile range (IQ25-75%), according to normality. The Pearson correlation coefficient was used to evaluate the linear association between the scores of the quality of life questionnaires (CRQ or SF36) (dependent variables) with psychosocial scale (HADS) scores, exercise tests (CPET, ISWT, and 6MWT) and lung function (PFTs) parameters (independent variables). The linear correlation (r) was considered weak (< 0.29), moderate (from 0.3 to 0.49), or strong (≥ 0.5)¹⁷. Multiple linear forward regression analysis was performed when the independent variables had a linear correlation (p < 0.2). The best predictive models were constructed using the best independent coefficient since there was no multicollinearity assessing the variance inflation factor (VIF < 2). The level of significance was set at 5% (p < 0.05). The data were analysed using Sigma Stat version 3.5 (Systat Software, Inc., San Jose, CA).

Sixty women were eligible and invited to participate, and 15 declined because they lived far from the hospital and could not attend the second appointment (Figure 1). Therefore, 45 women were included, and their clinical, anthropometric, and functional data are presented in Table 1. Only one woman was older than sixty-five years old. On average, the women were overweight and had good exercise capacity as assessed by 6MWT and ISWT (510 and 429 meters, 90% and 78% of the predicted, respectively). However, when exercise capacity was assessed by the CPET, 36 patients (86%) reached the peak VO₂ below 84% of the predicted value. Patients presented the lowest SF-36 scores in the general health and vitality domains and the highest in the physical and social role domains. Regarding the CRQ, the lowest scores were for dyspnoea and fatigue and the highest for the emotional function and self-control domains. Sixteen (35%) patients had anxiety, and 8 (17%) also had depression symptoms. Moreover, the obstructive pattern, air trapping, and reduced DLco were observed in 60%, 57%, and 15% of the patients, respectively (Table 1).

The associations of the generic and specific questionnaire results with the outcomes of physical capacity, PFTs, anxiety, and depression symptoms are shown in Tables 2 and 3. Regarding the generic questionnaire (SF-36), a strong association was observed between the physical functioning (r=0.53, p<0.001) and vitality (r=0.50, p<0.01) domains and physical capacity (peak VO₂ in ml.kg^-1.min^-1). The mental health domain also showed a strong association with anxiety and depression symptoms (r=-0.65, p≤0.0001 and r=-0.70, p<0.001, respectively). Moreover, symptoms of depression presented a moderate to strong association (ranging from r=0.30to r=0.70, p<0.05) with all the SF-36 domains. The DLco was the functional parameter that presented the strongest association with the physical functioning domain (r=0.43, p<0.003), whereas there was no association with FEV₁ or the RV/TLC. Regarding the specific questionnaire (CRQ), there was a moderate association between the dyspnoea (r=0.41, p=0.007) and fatigue (r=0.40, p=0.01) domains and physical capacity (peakVO₂, in ml.kg^-1.min^-1). The fatigue, emotional function, and self-control domains showed the strongest associations with the anxiety and depression domains (HADS A [r=-0.50, r=-0.62, and r=-0.60, respectively; p<0.001 for all the correlations] and HADS D [r=-0.55, r=-0.65 and r=-0.64; p<0.001 for all the correlations]). Air trapping (RV/TLC in % of predicted) was the functional parameter with better, although weak, association with the fatigue domain (r=-0.32, p=0.03). The DLco and FEV₁ variables, both in % of predicted, had no association with HRQoL and were not included in the regression models.

The associations between the SF-36 and CRQ domains and the symptoms of depression were very clear. All domains of the generic and specific questionnaires were correlated with physical capacity outcomes, anxiety, and depression symptoms, while only some were associated with lung function (p<0.2). All the data from the generic and specific questionnaires that were used for multiple linear regression modeling are presented in Table 4.

To the best of our knowledge, this is the first study to investigate the factors associated with HRQoL in LAM patients. Our results showed that general health and vitality were the most highly impacted domains assessed by the generic questionnaire (SF-36). Additionally, dyspnoea and fatigue were the most highly impacted domains assessed by the specific questionnaire (CRQ). Furthermore, physical capacity, anxiety, and depression symptoms were strongly associated with HRQoL. Finally, lung function parameters were weakly associated with HRQoL.

In the current study, we observed that general health and vitality were the most strongly impaired SF-36 domains. Interestingly, our observations are similar to those reported by Baldi and coworkers⁶ (general health: 56 ± 19 vs. 65 ± 25; vitality: 56 ± 20 vs. 64 ± 23). The National Heart, Lung, and Blood Institute provides the largest registry, enrolling 230 patients with LAM, and the physical component domain of the SF-36 was lower than the mental component score (39.7 ± 0.82 and 50.2 ± 0.66, respectively)¹⁸. These results emphasize that LAM patients have impaired quality of life, with worse scores in the physical and emotional domains when assessed using SF-36.

According to the HRQoL, as determined by a specific questionnaire (CRQ), dyspnoea and fatigue are the most strongly impaired domains. Our results are partially supported by a previous study demonstrating that dyspnoea and fatigue are reported by the vast majority of patients with interstitial lung disease (ILD)¹⁹. In addition, a CRQ validation study of Brazilian patients with COPD obtained scores similar to those of our LAM patients in all domains (fatigue 4.5 ± 1.2 vs. 4.3 ± 1.2; emotional function 4.8 ± 1.0 vs. 4.4 ± 1; self-control 5.1 ± 1.3 vs. 5.1 ± 1.1 score; respectively), except for the dyspnoea domain, which was worse in LAM patients than in patients with COPD (4.6 ± 1.3 vs. 3.8 ± 1.4 score, respectively). Given that a < 0.5-point difference in each domain is the minimum clinically important difference, we can assume that the quality of life of patients with LAM is quite similar to that of patients with severe to very severe COPD in most CRQ domains, despite the large difference in lung function between the two groups of patients²⁰. The discrepancy in the dyspnoea domain scores between LAM and COPD patients might be explained by the specific characteristics of each population, such as age and physical activity levels, as well as disease severity and the subjective aspects related to the perception of dyspnoea.

Martinez and coworkers²¹ reported that the general health and vitality domains assessed by the SF-36 had a good relationship with dyspnoea scores in patients with IPF. However, the outcomes associated with HRQoL in LAM patients remain poorly known, mainly because the disease is quite rare. Our results showed an association between the domains of generic and specific questionnaires and the physical capacity parameters as assessed by laboratory or field tests. The CPET is the gold standard for quantifying exercise capacity and evaluating the pathophysiological mechanisms of dyspnoea and exercise limitations in people with CRDs. In patients with COPD and IPF, aerobic capacity (VO₂, in kg/min) is a marker of mortality and induced hypoxemia and also an important marker to assess the response to pulmonary rehabilitation²². Our results clearly demonstrated that aerobic capacity (peak VO₂) and depression symptoms were the main variables independently associated with almost all SF-36 and CRQ domains. However, since this was a cross-sectional study, it is not possible to infer causality. Most women receive a diagnosis during a productive and reproductive period of life, and such a diagnosis may affect them, increasing the risk of developing anxiety and depression symptoms. Most likely, the increase in those symptoms may reduce their physical activity, impacting their aerobic capacity. As a consequence, it seems reasonable to assume that anxiety and depression symptoms and physical capacity are the most relevant features to LAM patient's quality of life.

We also observed that lung function variables were associated with few HRQoL domains, suggesting that treatments to improve patient's quality of life should be more focused on reducing anxiety and depression symptoms and improving exercise capacity than PFTs. In contrast to our results, previous studies have shown that PFTs are associated with quality of life as assessed by the SF-36 or CRQ in COPD patients²³. Moua and coworkers²⁴ also reported an association between FVC% pred and CRQ domains in patients with ILDs. However, it is difficult to compare these results appropriately because respiratory diseases differ due to their unique characteristics.

In a systematic review that evaluated features associated with HRQoL in patients with ILDs, the strongest correlation was observed between dyspnoea and the domains that concern physical health. On the other hand, the correlations between lung function parameters (FVC and DL_CO) or oxygenation and HRQoL domains were weaker²⁵. Based on our findings in which anxiety and depression symptoms remained independent outcomes associated with all HRQoL domains, we suggest that physicians should pay more attention to patients' psychosocial assessment.

Moreover, anxiety and depression symptoms occur in approximately 25% of patients with ILDs, and the percentages of these patients with clinically meaningful depression range from 7 to 49%, and with anxiety from 9 to 12%^26,27, however, the percentage of patients with those symptoms is even greater among LAM patients, reaching 53%^26,28. Depression and anxiety symptoms are not only essential in predicting the HRQoL of ILD patients but can also be associated with breathlessness levels²⁸. Therefore, routine screening for depression and other underlying symptoms that can increase psychological stress and may decrease patients' HRQoL should be performed⁴⁵. The present study showed that symptoms of anxiety and depression were present in LAM patients, which may lead to a vicious cycle because the greater their levels, the more physically limited patients may become, impacting the quality of life of even more individuals.

Our study had several limitations. First, it was performed in a single centre. However, our centre is the primary for treating LAM in Latin America and we assist patients from all regions of Brazil with different severities of disease. In addition, 45 participants can be considered a significant sample size due to the rarity of the disease Second, we excluded patients using continuous oxygen because their mobility is usually reduced. Therefore, our results cannot be extrapolated to this subgroup with worse disease severity. Third, there is no specific validated HRQoL questionnaire for LAM, and the CRQ, which is the most used disease-specific tool for assessing HRQoL in patients with CRDs¹¹, was used for this study.

Our results showed that aerobic capacity and depression symptoms are the principal factors associated with the quality of life of LAM patients; however, lung function parameters were poorly associated. These findings might alert clinicians to the need to conduct frequent psychosocial and physical exercise assessments for future intervention to improve the quality of life of LAM patients. Further studies are required to identify a questionnaire that better assesses the quality of life of this population.

Funding information

The study was supported by grants 312279/2018-3 from the Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) and grants 2018/17788-3 from Fundação de Amparo à Pesquisa de São Paulo (FAPESP)

Author Contribution

DQ promoted the development of the study design, the scheduling of patient appointments, data collection and analysis, and interpreting the data, as well as writing the article. CS assisted in their search, helping develop the study design, scheduling patient appointments, collecting, analyzing, and interpreting the data, and improving and developing the article. MO and AA promoted the development of the study design and the scheduling of patient appointments. CRRC greatly contributed to developing the study design, analyzing and interpreting the data, and helping with the elaboration of the article. JMS promoted the development of the study design and the scheduling of patient appointments. BB supported us by developing the study design, scheduling patient appointments, analyzing and interpreting the data, and helping with the later elaboration of the article. CRFC conducted our research and provided insight and expertise in all stages of the study, from the concept to the design, data collection and analysis, data interpretation, improvements, and the development of the article. All authors contributed to the article and approved the version he submitted.

Data Availability

Sequence data that support the findings of this study have been deposited in the Zenodo with the primary accession code md5:166d8ecdbfd8d5c566b4b3bc07d2f995

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Tables 1 to 4 are available in the Supplementary Files section.

No competing interests reported.

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Clinical and functional outcomes associated with quality of life in patients with lymphangioleiomyomatosis: a cross-sectional study

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