Prospective Cohort of Patients With Fibrosing Interstitial Lung Disease Admitted for Acute Respiratory Failure

This prospective, multicenter study focuses on patients with fibrosing interstitial lung diseases (ILD) admitted to intensive care units (ICUs) for acute respiratory failure (ARF). ILD encompasses a group of heterogeneous disorders characterized by progressive fibrotic and inflammatory damage to the lung interstitium, often resulting in chronic respiratory failure. Acute respiratory failure in these patients is a severe and frequent complication caused by various factors, including infections, pulmonary embolism, cardiac decompensation, or acute exacerbation of fibrosis. Despite advances in treatment, ICU management of these patients remains a clinical and ethical challenge due to high mortality rates and limited evidence-based guidance.

The study's primary objective is to assess 6-month survival following ICU admission for ARF in ILD patients. Secondary objectives include identifying prognostic factors for mortality, characterizing ICU and hospital mortality rates, and evaluating the use of organ support measures (e.g., oxygen therapy, invasive or non-invasive mechanical ventilation). Additionally, the study will examine the frequency of lung transplantation, acquired ICU infections, and decisions to limit or withdraw life-sustaining therapies.

Importantly, this research aims to address a significant gap in current knowledge by building a large, descriptive, prospective cohort of ILD patients. A critical aspect of the study is the evaluation of patients' quality of life (QoL) six months after ICU discharge, using a dedicated questionnaire. This information will provide essential insights into the long-term benefits of ICU care for these patients, complementing survival data with QoL outcomes to better guide clinical decision-making and improve patient-centered care.