Article Text
Abstract
Background Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias and is characterised by progressive accumulation of scar tissue in the lungs. It carries a poor prognosis, with a median survival of only approximately 3 years after diagnosis. No previous reports have attempted to describe trends in IPF mortality across the European Union (EU).
Methods Country-level data for IPF mortality, identified in the WHO mortality database using ICD-10 codes for the period 2001–2013. Joinpoint analysis was performed to describe trends throughout the observation period.
Findings Median mortality was 3.75/1 00 000 (IQR, 1.37–5.30) and 1.50/1 00 000 (IQR, 0.65–2.02) for males and females, respectively. IPF mortality increased in the majority of the EU countries with the exceptions of Denmark, Croatia, Austria and Romania. There was a significant disparity in rates across Europe ranging from 0.41–12.1/100 000 for men and 0.24–5.63/100 000 for women. Most notable increases were observed in the UK and Finland. Rates were also substantially higher in males with gender disparity increasing across the period.
Interpretation Reported IPF mortality appears to be increasing across the EU, however there is substantial variation in mortality trends and overall reported mortality rates between countries. There are likely to be differences in coding practices and reporting levels between countries, particularly with specialist knowledge and equipment required to diagnose IPF.