Sleep desaturation and its relationship to lung function, exercise and quality of life in LAM

Medeiros, Paulo José de; Lorenzi-Filho, Geraldo; Pimenta, Suzana Pinheiro; Kairalla, Ronaldo Adib; Carvalho, Carlos Roberto Ribeiro

doi:10.1016/j.rmed.2011.12.008

Cited by 13 publications

(4 citation statements)

References 36 publications

(18 reference statements)

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“…12 Desaturation during sleep has been well studied in chronic respiratory diseases such as COPD and lymphangioleiomyomatosis (LAM), but it has been poorly studied in IPF. 40,41 It was shown that patients with mild daytime hypoxemia may be particularly vulnerable to desaturation during sleep because they often reside on the steep portion of the oxyhemoglobin dissociation curve. COPD-OSA overlap syndrome patients have more pronounced nocturnal hypoxemia and are at increased risk of death.…”

Section: Discussionmentioning

confidence: 99%

Sleep Oxygen Desaturation Predicts Survival in Idiopathic Pulmonary Fibrosis

Kolilekas

Manali

Vlami

et al. 2013

Journal of Clinical Sleep Medicine

121

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background: Recent studies suggest poor sleep quality in patients with idiopathic pulmonary fi brosis (IPF). However, so far, the impact of IPF-related sleep breathing disorders (SBDs) on survival has not been extensively studied. Methods: In a cohort of 31 (24 males) treatment-naïve, newly diagnosed consecutive IPF patients, we prospectively investigated the relationship of SBD parameters such as apnea-hypopnea index (AHI), maximal difference in oxygen saturation between wakefulness and sleep (maxdiff SpO 2 ), and lowest sleep oxygen saturation (lowest SpO 2 ) with clinical (survival, dyspnea, daytime sleepiness), pulmonary function, submaximal (6-min walk test [6MWT]) and maximal exercise variables (cardiopulmonary exercise test [CPET]), and right ventricular systolic pressure (RVSP). Results: Sleep oxygen desaturation exceeded signifi cantly that of maximal exercise (p < 0.001). Maxdiff SpO 2 was inversely related to survival, DLCO%, and SpO 2 after 6MWT, and directly with dyspnea, AHI, and RVSP. The lowest SpO 2 was directly related to survival and to functional (TLC%, DLCO%) as well as submaximal and maximal exercise variables (6MWT

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Section: Discussionmentioning

confidence: 99%

Sleep Oxygen Desaturation Predicts Survival in Idiopathic Pulmonary Fibrosis

Kolilekas

Manali

Vlami

et al. 2013

Journal of Clinical Sleep Medicine

121

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“…Dyspnea and lower exercise capacity may be associated with several factors, such as dynamic hyperinflation, worsening gas exchange and, potentially, pulmonary hypertension (PH). Hypoxemia may occur at rest, during exercise or even during sleep, mainly in those patients with a higher degree of impairment in pulmonary function tests (PFTs) [5, 7, 8]. …”

Section: Introductionmentioning

confidence: 99%

Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method

Freitas

Baldi

Jardim

et al. 2017

Orphanet J Rare Dis

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BackgroundLymphangioleiomyomatosis (LAM) is included within group 5 of the current PH classification (unclear multifactorial mechanisms). However, data regarding the occurrence of PH in LAM are scarce. The aims of the study were to describe the prevalence and characteristics of PH in a large cohort of LAM patients with different levels of severity, and to evaluate the role of echocardiography and carbon monoxide diffusion capacity (DLCO) as screening methods for PH in LAM.MethodsOne hundred five LAM patients underwent transthoracic echocardiography, pulmonary function tests (PFTs) and 6-min walk test (6MWT). Patients with a suspicion of PH on echocardiography, defined by the presence of estimated systolic pulmonary artery pressure (PAP) over 35 mmHg or PFT showing DLco below 40% of the predicted value, underwent right heart catheterisation to confirm the diagnosis of PH.ResultsEight patients (7.6%) had PH confirmed on right heart catheterisation, six patients (5.7%) had a pre-capillary pattern and two patients (1.9%) had a post-capillary profile. Only one patient (1%) had mean PAP over 35 mmHg. Patients with PH had lower FEV1 and DLCO in PFTs and greater oxygen desaturation and dyspnea intensity during 6MWT compared with those without PH. In 63% of the patients with confirmed PH, the right heart catheterisation was performed based only on DLCO result.ConclusionsThe prevalence of PH is low in LAM patients. Pulmonary hypertension in LAM is typically mild and significantly associated with pulmonary parenchymal involvement. Carbon monoxide diffusion capacity significantly improved the identification of PH in LAM patients.

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“…Pulmonary function tests (PFTs) show an obstructive pattern and reduced diffusion capacity [ 12 ]. Hypoxemia during sleep is a common finding [ 13 ]. Chest X-Ray is normal in most patients with LAM unless there is a complication due to pleural effusions or pneumothorax.…”

Section: Discussionmentioning

confidence: 99%

Pregnancy unmasking symptoms of undiagnosed lymphangioleiomyomatosis: Case report and review of literature

Khaddour

Shayuk

Ludhwani

et al. 2019

Respiratory Medicine Case Reports

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Cystic lung diseases are a group of disorders that appear similar on radiological studies on chest computed tomography. Each disorder is characterized by its own etiology, pathophysiology, course of progression and manifestation. Lymphangioleiomyomatosis (LAM) is one of the cystic lung diseases that can either be hereditary or sporadic. The sporadic form is a rare disease with no accurate prevalence reported but is believed to be less than 10 per million. LAM is associated with inappropriate activation of mammalian target of rapamycin (mTOR) signaling which regulates cellular growth. The sporadic form is almost confined to premenopausal female population and estrogen is believed to play an important role in the pathogenesis. Pregnancy and use of estrogen based oral contraceptives can aggravate symptoms of already existing LAM. Here we describe a case of LAM that was previously treated as asthma and was diagnosed after exacerbation of respiratory symptoms after pregnancy. We offer a review of the medical literature regarding the etiology, clinical course, diagnosis and treatment of LAM.

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Sleep desaturation and its relationship to lung function, exercise and quality of life in LAM

Abstract: Significant hypoxaemia during sleep is common in LAM patients with normal SpO(2) while awake, especially among those with some degree of hyperinflation in lung function tests.

Cited by 13 publications

References 36 publications

Sleep Oxygen Desaturation Predicts Survival in Idiopathic Pulmonary Fibrosis

Sleep Oxygen Desaturation Predicts Survival in Idiopathic Pulmonary Fibrosis

Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method

Pregnancy unmasking symptoms of undiagnosed lymphangioleiomyomatosis: Case report and review of literature

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