Clinical experience in idiopathic pulmonary fibrosis: a retrospective study

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is a rare lung disease with an increased incidence since the last few years. Here, we report our eight-year clinical experience in CHU of Liège, Belgium. Methods: We have studied retrospectively patients recruited from our ambulatory care polyclinic at CHU of Liège from 1 January 2009 to 1 January 2017. We have excluded all patients treated with a specific anti-fibrotic therapy due to incomplete follow-up. The diagnosis of IPF was made according to the ATS/ERS international recommendations (2015). Results: Out of the 114 patients initially selected, 82 cases were found to be suitable for the analysis. The average age was 71.1 ± 9.35 years with a male predominance. The median survival was 43.7 months (23.6-71.7) with a majority (45%) of patients in the group II of the GAP index. The median rate of annual decline in diffusion capacity of CO (DLCO) was 11%, whereas the sub analysis for group III (according to GAP index) showed a decrease annual rate of 30%. Conclusion: Our results are in keeping with the literature. One of our major finding is that patients in GAP III exhibit an annual rate of mortality of 42% and a median annual decline in DLCO of 30%. This observation highlights the fact that this specific subgroup of patients presents a high risk of morbi-mortality.

Macrophage-derived exosomes attenuate fibrosis in airway epithelial cells through delivery of antifibrotic miR-142-3p

“…Two independent study populations were enrolled at the ambulatory care polyclinics of Liège University. The diagnosis of (definite) IPF was made according to the international recommendations of the American Thoracic Society/European Respiratory Society 34 (more information in online supplementary methods). The first cohort of 19 patients with IPF and 23 HSs was used to study the alteration of exosomal miR levels in the sputum, whereas the second cohort of 14 patients with IPF and 14 HSs was used to study the alteration of exosomal miR levels in the plasma.…”

Exosomal miRNAs in Lung Diseases: From Biologic Function to Therapeutic Targets

“…IPF is a progressive fibrosing interstitial lung disease of unknown etiology and cure which leads to rapid death within 2–3 years after diagnosis [56,57,58,59,60,61]. IPF is characterized by progressive and irreversible destruction of the lung architecture caused by fibrotic “scar” formation that ultimately leads to organ destruction and death from respiratory failure [62,63].…”

Serum IGFBP-2 in Systemic Sclerosis as a Prognostic Factor of Lung Dysfunction

“…Lung brosis is present in approximately 25% of SSc patients [13]. Contrary to what is seen in IPF [14], treatment is mainly based on an aggressive immunosuppressive therapy speci cally proposed in the progressive forms of SSc-ILD [15,16]. One of the major problem clinicians have to deal with is to identify patients with increased risk of ILD progression for early intervention [6,[17][18][19][20].…”