Inspiratory Effort and Respiratory Mechanics in Spontaneously Breathing Patients With Acute Exacerbation of Idiopathic Pulmonary Fibrosis: a Matched Control Study

NCT ID: NCT05313672

Last Updated: 2025-04-25

Basic Information

• Recruitment Status: ACTIVE_NOT_RECRUITING
• Total Enrollment: 20 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2016-08-01
• Study Completion Date: 2026-08-01

Brief Summary

Idiopathic pulmonary fibrosis is a life-threatening lung disease characterized by progressive deterioration of lung function and a median survival time of 3-5 years from diagnosis. The onset of an acute deterioration (AE) of respiratory function, the so called acute exacerbation of IPF (AE-IPF), may lead to severe hypoxemia, further worsening prognosis. During these events, the typical usual interstitial pneumonia pattern (UIP) - the radiologic and histologic hallmark of IPF- is overlapped with diffuse alveolar damage (DAD), sharing similarities with the acute respiratory distress syndrome (ARDS) and often requiring respiratory assistance. Several studies show that the need for mechanical ventilation (MV) is associated with high mortality in IPF patients, probably due to the pathophysiological properties of UIP-like fibrotic lung (i.e. collapse induration areas, elevated lung elastance, high inhomogeneity) that make it more susceptible to ventilatory-induced lung injury (VILI). It has been theorized that the application of PEEP on a UIP-like lung pattern can determine the protrusion of the most distensible areas through a dense anelastic fibrotic tissue circles, causing increased rigidity, worsening compliance, and thus enabling tissue breakdown. In this scenario, non-invasive mechanical ventilation (NIV) may therefore represent an alternative option to assist these patients, although no specific recommendations have been made so far. In patients with ARDS, the efficacy of NIV in reducing the patient's inspiratory effort early after its application has been related to a favorable clinical outcome. Indeed, the mitigation of respiratory drive might have resulted in a lower risk for the self-inflicted lung injury (SILI) during spontaneous breathing, whose onset is very likely to worse outcomes of patients undergoing acute respiratory failure (ARF).

To date no data available on the inspiratory effort and the lung mechanics in patients with AE-IPF either during unassisted of assisted spontaneous breathing. Aim of this study was then to compare respiratory mechanics, at baseline and 2-h following NIV application, in AE-IPF and ARDS patients matched for severity.

Conditions

Idiopathic Pulmonary Fibrosis

Study Design

• Observational Model Type: CASE_CONTROL
• Study Time Perspective: RETROSPECTIVE

Study Groups

IPF patients with UIP pattern requiring NIV

No interventions assigned to this group

ARDS patients requiring NIV

No interventions assigned to this group

Eligibility Criteria

Inclusion Criteria

previously established diagnosis of IPF; occurring acute exacerbation of IPF as defined by an acute, clinically significant respiratory deterioration characterized by evidence of new widespread alveolar abnormality on chest CT scan (12); age >18 years; presence of ARF with PaO2/FiO2 ratio <200 mmHg despite HFNC (set with at least 60 L/min and FiO2 higher than 0.6); suitability for a NIV trial according to the attending staff; consent to measure esophageal pressure.

Exclusion Criteria

• acute cardiogenic pulmonary edema,
• concomitant hypercapnic respiratory failure (PaCO2 >45 mmHg) of any etiology,
• neuromuscular disease or chest wall deformities,
• home long-term oxygen therapy,
• intolerance or contraindication to NIV, such as the need for immediate endotracheal intubation

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Roberto Tonelli

OTHER

Sponsor Role: lead

Responsible Party

Roberto Tonelli

Principal investigator

Responsibility Role: SPONSOR_INVESTIGATOR

Other Identifiers

UModenaReggio21

Identifier Type: -

Identifier Source: org_study_id