In search of the fibrotic epithelial cell: opportunities for a collaborative network

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive
disease of unknown aetiology. It has a very poor prognosis and no
effective treatment. There are two major barriers to the
development of novel treatments in IPF: an incomplete understanding
of its pathogenesis and the fact that current models of the disease
are poorly predictive of therapeutic response. Recent studies
suggest an important role for the alveolar epithelium in the
pathogenesis of IPF. However, practical limitations associated with
isolation and culture of primary alveolar epithelial cells have
hampered progress towards further elucidating their role in the
pathogenesis of the disease or developing disease models that
accurately reflect the epithelial contribution. The practical
limitations of primary alveolar epithelial cell culture can be
divided into technical, logistical and regulatory hurdles that need
to be overcome to ensure rapid progress towards improved treatment
for patients with IPF. To develop a strategy to facilitate alveolar
epithelial cell harvest, retrieval and sharing between IPF research
groups and to determine how these cells contribute to IPF, a
workshop was organised to discuss the central issues surrounding
epithelial cells in IPF (ECIPF). The central themes discussed in
the workshop have been compiled as the proceedings of the ECIPF.