Management of Chronic Respiratory Failure in Interstitial Lung Diseases: Overview and Clinical Insights

Abstract

Interstitial lung diseases (ILDs) may be complicated by chronic respiratory failure (CRF), especially in the advanced stages. Aim of this narrative review is to evaluate the current evidence in management of CRF in ILDs. Many physiological mechanisms underlie CRF in ILDs, including lung restriction, ventilation/perfusion mismatch, impaired diffusion capacity and pulmonary vascular damage. Intermittent exertional hypoxemia is often the initial sign of CRF, evolving, as ILD progresses, into continuous hypoxemia. In the majority of the cases, the development of CRF is secondary to the worsening of the underlying disease; however, associated comorbidities may also play a role. When managing CRF in ILDs, the need for pulmonary rehabilitation, the referral to lung transplant centers and palliative care should be assessed and, if necessary, promptly offered. Long-term oxygen therapy is commonly prescribed in case of resting or exertional hypoxemia with the purpose to decrease dyspnea and improve exercise tolerance. High-Flow Nasal Cannula oxygen therapy may be used as an alternative to conventional oxygen therapy for ILD patients with severe hypoxemia requiring both high flows and high oxygen concentrations. Non-Invasive Ventilation may be used in the chronic setting for palliation of end-stage ILD patients, although the evidence to support this application is very limited.

Comparison of SpO2 and heart rate values on Apple Watch and conventional commercial oximeters devices in patients with lung disease

“…As in COPD, oxygen therapy is also instituted in most cases of patients with interstitial lung diseases (ILD) 16 , 17 . This is a group of lung diseases characterized by alveolar damage with fibroblastic and/or inflammatory proliferation.…”

Hyperbaric Oxygen Ameliorates Bleomycin-Induced Pulmonary Fibrosis in Mice

“…For patients with advanced disease, reducing symptoms and improving quality of life are required (Zou et al, 2020). Long-term oxygen therapy, with high flow and high concentration of oxygen, is often used to decrease dyspnea and improve exercise tolerance (Koyauchi et al, 2018;Faverio et al, 2019). It is also reported that oxygen supplementation increased exercise capacity for patients with interstitial lung diseases including IPF (Bell et al, 2017;Dowman et al, 2017).…”

Non-invasive Ventilation for Children With Chronic Lung Disease

“…Continuous PAP provides alveolar inflation and prevents atelectasis. The physiological benefits of CPAP such as stabilization of chest wall and upper airway, reduction of lung resistance and improvement of tidal volume, oxygenation, and functional residual capacity increase the success of extubation of preterm infants ( 9 ). The use of CPAP significantly reduced the need for invasive mechanical ventilation but the rates of CPAP failure are high.…”

“…While surfactant protein deficiency, neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenesis, and developmental disorders are specific to infancy, ILD related to exposure/environment insults, ILD related to systemic and immune diseases, and ILD related to primary lung parenchyma dysfunctions constitute the non-age specific group ( 88 ). Anti-inflammatory and immunosuppressive agents are the cornerstones of pharmacological therapy, while oxygen and ventilatory support are required for patients with hypoxemia and respiratory failure ( 9 ).…”

“…In addition, increased lung resistance and work of breathing result in increased respiratory rate. An impairment in gas exchange due to ventilation-perfusion mismatch and reduced diffusion capacity also contribute to the increase in respiratory rate and respiratory load ( 8 , 9 ).…”