Nocturnal Hypoxemia Associates With Symptom Progression and Mortality in Patients With Progressive Fibrotic Interstitial Lung Disease

Myall, Katherine; West, Alex; Martinovic, Jennifer L.; Lam, Jodie L; Roque, Dana M.; Wu, Zhe; Maher, Toby M.; Molyneaux, P.L.; Suh, Eui‐Sik; Kent, Brian D.

doi:10.1016/j.chest.2023.05.013

Cited by 6 publications

(2 citation statements)

References 31 publications

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“…Approximately one-third of patients newly diagnosed with fibrotic ILD have evidence of obstructive sleep apnea on polysomnography . There is no evidence to date that treating the obstructive sleep apnea alters prognosis . Approximately 14% of individuals newly diagnosed with IPF have pulmonary hypertension, and this number increases to approximately 86% in individuals with pulmonary fibrosis awaiting lung transplant .…”

Section: Epidemiologymentioning

confidence: 99%

Interstitial Lung Disease

Maher

2024

JAMA

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ImportanceInterstitial lung disease (ILD) consists of a group of pulmonary disorders characterized by inflammation and/or fibrosis of the lung parenchyma associated with progressive dyspnea that frequently results in end-stage respiratory failure. In the US, ILD affects approximately 650 000 people and causes approximately 25 000 to 30 000 deaths per year.ObservationsThe most common forms of ILD are idiopathic pulmonary fibrosis (IPF), which accounts for approximately one-third of all cases of ILD, hypersensitivity pneumonitis, accounting for 15% of ILD cases, and connective tissue disease (CTD), accounting for 25% of ILD cases. ILD typically presents with dyspnea on exertion. Approximately 30% of patients with ILD report cough. Thoracic computed tomography is approximately 91% sensitive and 71% specific for diagnosing subtypes of ILDs such as IPF. Physiologic assessment provides important prognostic information. A 5% decline in forced vital capacity (FVC) over 12 months is associated with an approximately 2-fold increase in mortality compared with no change in FVC. Antifibrotic therapy with nintedanib or pirfenidone slows annual FVC decline by approximately 44% to 57% in individuals with IPF, scleroderma associated ILD, and in those with progressive pulmonary fibrosis of any cause. For connective tissue disease–associated ILD, immunomodulatory therapy, such as tocilizumab, rituximab, and mycophenolate mofetil, may slow decline or even improve FVC at 12-month follow-up. Structured exercise therapy reduces symptoms and improves 6-minute walk test distance in individuals with dyspnea. Oxygen reduces symptoms and improves quality of life in individuals with ILD who desaturate below 88% on a 6-minute walk test. Lung transplant may improve symptoms and resolve respiratory failure in patients with end-stage ILD. After lung transplant, patients with ILD have a median survival of 5.2 to 6.7 years compared with a median survival of less than 2 years in patients with advanced ILD who do not undergo lung transplant. Up to 85% of individuals with end-stage fibrotic ILD develop pulmonary hypertension. In these patients, treatment with inhaled treprostinil improves walking distance and respiratory symptoms.Conclusions and RelevanceInterstitial lung disease typically presents with dyspnea on exertion and can progress to respiratory failure. First-line therapy includes nintedanib or pirfenidone for IPF and mycophenolate mofetil for ILD due to connective tissue disease. Lung transplant should be considered for patients with advanced ILD. In patients with ILD, exercise training improves 6-minute walk test distance and quality of life.

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Section: Epidemiologymentioning

confidence: 99%

Interstitial Lung Disease

Maher

2024

JAMA

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“…This is the largest study examining nocturnal hypoxia in the population of ILD to date, providing new evidence in this area. A recent prospective observational cohort study from the UK (recruited from 2018 to 2020, mainly IPF) also found that nocturnal hypoxia is associated with a more rapid decline in the quality of life and increased 1-year all-cause mortality [ 8 ]. These studies underscore the urgent need for intervention in nocturnal hypoxia.…”

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confidence: 99%

The “silent threat” of nocturnal hypoxia remains unresolved for patients with fibrotic interstitial lung diseases

Suzuki

2024

ERJ Open Res

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Extract Sleep is an inherent and vital state of rest for both the body and mind. While it is recommended that adults get 7–8 h of sleep, patients with fibrotic interstitial lung disease (ILD) often experience poor sleep quality and shorter sleep duration [1–4]. Notably, nocturnal hypoxia is widely recognised as a comorbidity that occurs during sleep [5]. The most significant issue with nocturnal hypoxia is not only its impact on worsening sleep quality but also its contribution to the deterioration of pulmonary hypertension and the resulting poor prognosis [5]. Despite the acknowledgement of this issue by many clinicians and researchers, the management still lacks large-scale clinical trials, resulting in a lack of clear recommendations [6].

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