Int J Med Sci 2019; 16(7):967-980. doi:10.7150/ijms.32752 This issue


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

Paola Faverio1, Federica De Giacomi1, Giulia Bonaiti1, Anna Stainer1, Luca Sardella1, Giulia Pellegrino2, Giuseppe Francesco Sferrazza Papa2, Francesco Bini3, Bruno Dino Bodini4, Mauro Carone5, Sara Annoni6, Grazia Messinesi1, Alberto Pesci1✉

1. School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy; Respiratory Unit, San Gerardo Hospital, ASST di Monza, Monza, Italy
2. Casa di Cura del Policlinico, Dipartimento di Scienze Neuroriabilitative, Milan, Italy
3. UOC Pulmonology, Department of Internal Medicine, Ospedale ASST-Rhodense, Garbagnate Milanese, Italy
4. Pulmonology Unit, Ospedale Maggiore della Carità, University of Piemonte Orientale, Novara, Italy
5. UOC Pulmonology and Pulmonary Rehabilitation, Istituti Clinici Scientifici Maugeri, IRCCS di Cassano Murge (BA), Italy
6. Physical therapy and Rehabilitation Unit, San Gerardo Hospital, ASST di Monza, Monza, Italy.

This is an open access article distributed under the terms of the Creative Commons Attribution (CC BY-NC) license ( See for full terms and conditions.
Faverio P, De Giacomi F, Bonaiti G, Stainer A, Sardella L, Pellegrino G, Sferrazza Papa GF, Bini F, Bodini BD, Carone M, Annoni S, Messinesi G, Pesci A. Management of Chronic Respiratory Failure in Interstitial Lung Diseases: Overview and Clinical Insights. Int J Med Sci 2019; 16(7):967-980. doi:10.7150/ijms.32752. Available from

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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, idiopathic pulmonary fibrosis, chronic respiratory failure, non-invasive ventilation, oxygen therapy