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.

Keywords:

• Idiopathic pulmonary fibrosis
• interstitial lung diseases
• prognosis