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Review
. 2019 Jun 10;16(7):967-980.
doi: 10.7150/ijms.32752. eCollection 2019.

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

Paola Faverio  1 , Federica De Giacomi  1 , Giulia Bonaiti  1 , Anna Stainer  1 , Luca Sardella  1 , Giulia Pellegrino  2 , Giuseppe Francesco Sferrazza Papa  2 , Francesco Bini  3 , Bruno Dino Bodini  4 , Mauro Carone  5 , Sara Annoni  6 , Grazia Messinesi  1 , Alberto Pesci  1
Affiliations
Review

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

Paola Faverio et al. Int J Med Sci. .

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.

Keywords: Interstitial lung diseases; chronic respiratory failure; idiopathic pulmonary fibrosis; non-invasive ventilation; oxygen therapy.

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Conflict of interest statement

Competing Interests: The authors have declared that no competing interest exists.

Figures

Figure 1
Figure 1
Diagnostic work-up of chronic respiratory failure in interstitial lung diseases. Footnotes: 6MWT: six minute walking test; CPFE: combined pulmonary fibrosis and emphysema; CRF: chronic respiratory failure; CT: computed tomography; DLCO: diffusing lung capacity for carbon monoxide; FVC: forced vital capacity; HF: heart failure; HRCT: high resolution computed tomography; ILD: interstitial lung disease; NT-proBNP: N-terminal pro B-type natriuretic peptide; OSAS: obstructive sleep apnea syndrome; PE: pulmonary embolism; PFTs : pulmonary function tests; PH: pulmonary hypertension; RHC: right heart catheterization.
Figure 2
Figure 2
Causes of chronic respiratory failure in interstitial lung diseases and when to suspect them. Footnotes: CAD: coronary artery disease; COPD: chronic obstructive pulmonary disease; CTD: connective tissue disease; CRF: chronic respiratory failure; DLCO: diffusing lung capacity for carbon monoxide; FVC: forced vital capacity; HRCT: high resolution computed tomography; ILD: interstitial lung disease; LES: Systemic lupus erythematosus; PE: pulmonary embolism; PFTs: pulmonary function tests; PH: pulmonary hypertension; TLC: total lung capacity.
Figure 3
Figure 3
Management and therapeutic options in interstitial lung diseases patients with chronic respiratory failure. Footnotes: CRF: chronic respiratory failure.
See this image and copyright information in PMC

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