The field of ILD has gone through several years of change.
Many old views on these diseases have been challenged and
replaced by more convincing concepts. A good example is the
change IPF therapy has undergone during recent years.
According to the US philosopher Thomas S. Kuhn (1922–1996),
progress in science often undergoes paradigmatic shifts rather
than continuous progress. In clinical medicine, however, many
examples show that continuous progress results in improved
diagnosis or therapy. The field of ILD and IPF is somehow a
mixture of both paradigm shift and continuous progress.
Ongoing research in recent decades provided insight into
disease mechanisms and underscored the heterogeneity and
complexity of these diseases. A chance paradigmatic shift in IPF
therapy followed the “crisis” finding that classical therapy with
immunosuppressants was more dangerous than beneficial. In
parallel, novel drugs were approved after their efficacy has been
demonstrated in clinical trials.
IPF is also a good example of a rare disease that needs special
attention in the diagnostic process. However, if IPF is taken
together with other ILDs, quite a high number of patients suffer
from these conditions, meaning the whole disease area goes far
beyond “rare” status. Interestingly, this field also gained much
more attention when novel drugs became available; pharmaceutical
advertisements in this field certainly increased.
The diagnosis and treatment of IPF is multidisciplinary, a fact
that is well represented by the content and authors of this book.
The book comprises chapters on molecular mechanisms,
diagnosis, imaging, pathology and various aspects of therapy. It
also reviews the fast developments in drug development. The
critical use of data from clinical trials provides new ways to
think about IPF and related diseases, and the shifted paradigms
of recent years. The Guest Editors, Ulrich Costabel, Bruno
Crestani and Athol U. Wells, have worked hard and very
successfully to select these topics and to integrate these aspects
Copyright ©ERS 2016. Print ISBN: 978-1-84984-067-5. Online ISBN: 978-1-84984-068-2. Print ISSN: 2312-508X. Online ISSN: 2312-5098.
ERS Monogr 2016; 71: v–vi. DOI: 10.1183/2312508X.10003316 v