Implementation of a standardized screening program for IPF patients and relatives in Flanders (IPF)

Idiopathic pulmonary fibrosis (IPF) is the most frequent form of
interstitial lung disease, essentially being a fibrotic scarring of the lung.
This fibrotic scarring of the lung leads to deterioration of pulmonary
function and is usually rapidly progressive, ultimately leading to patient
death with 3-5 years following diagnosis. IPF is believed to be the endresults
from a complicated interaction between multiple genetic and
non-genetic factors including older age, male sex, cigarette smoking,
environmental exposure, infectious agents, gastroesophageal reflux,
auto-immunity and a genetic predisposition. It is assumed that at least
one- third of the risk for developing IPF is explained by common gene
variants. Given its rapid progression, early detection, which is currently
not possible, is of crucial importance.
To achieve this, we propose to develop a multichannel screening
program for patients with an elevated risk for IPF being first line
relatives. For this purpose, in patients with a family history of IPF, IPF
patients will be subjected to a genetic screening program and relative
telomere length assessment. In addition, first line relatives will undergo
a combination of pulmonary function, chest CT scan, genetic screening
and relative telomere length assessment to identify early signs of
fibrotic lung disease and to assess their risk of developing IPF in the
future.
This project will therefore provide us with much needed answers on the
need and efficacy of screening programs and the optimal tools or
combination of tools to perform screening in a population at high risk
for developing this devastating disease. Moreover, it will provide clear
answers on whether a relative oriented or patient oriented screening
program is more effective.
Earlier detection of IPF will lead to earlier adequate treatment, which will preserve pulmonary function, improve quality of life and survival.