Risk factors for mortality and mortality rates in interstitial lung disease patients in the intensive care unit

Abstract

Data on interstitial lung disease (ILD) outcomes in the intensive care unit (ICU) is of limited value due to population heterogeneity. The aim of this study was to examine risk factors for mortality and ILD mortality rates in the ICU.We performed a systematic review using five databases. 50 studies were identified and 34 were included: 17 studies on various aetiologies of ILD (mixed-ILD) and 17 on idiopathic pulmonary fibrosis (IPF). In mixed-ILD, elevated APACHE score, hypoxaemia and mechanical ventilation are risk factors for mortality. No increased mortality was found with steroid use. Evidence is inconclusive on advanced age. In IPF, evidence is inconclusive for all factors except mechanical ventilation and hypoxaemia. The overall in-hospital mortality was available in 15 studies on mixed-ILD (62% in 2001–2009 and 48% in 2010–2017) and 15 studies on IPF (79% in 1993–2004 and 65% in 2005–2017). Follow-up mortality rate at 1 year ranged between 53% and 100%.Irrespective of ILD aetiology, mechanical ventilation is associated with increased mortality. For mixed-ILD, hypoxaemia and APACHE scores are also associated with increased mortality. IPF has the highest mortality rate among ILDs, but since 1993 the rate appears to be declining. Despite improving in-hospital survival, overall mortality remains high.

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“…Collagen Tissue Disease–associated Interstitial Lung Fibrosis (CTD-ILDs) and Bronchiolitis Obliterans Syndrome (BOS) represent two severe lung fibrogenic disorders involving, respectively, the lung interstitium and small airways, which share several pathogenic mechanisms and a poor long term outcome [1,2]. Common pathogenic steps include injury of airway epithelium due to immune-inflammatory insults, followed by a trigger of alveolar and bronchiolar epithelial cells transition towards myofibroblasts (epithelial mesenchymal transition, EMT) and a fibro-proliferative phase with uncontrolled extracellular matrix (ECM) deposition.…”

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