Idiopathic pulmonary fibrosis (IPF) is a chronic disease affecting the lungs and causing progressive loss of pulmonary function, ultimately leading to respiratory failure and death. There is no known cause for IPF and some genetic factors play a role in disease susceptibility.
This disease represents the most frequent and the most severe form of a group of various lung diseases, known as interstitial lung diseases.
Until recently, there were no therapies approved to be used in patients with IPF, so the management of these patients has been (and still is) a real challenge for respiratory physicians. Now, this has changed, with two drugs approved and recommended by guidelines for use in these patients. However, even in the new era of approved and safe treatments for IPF, patients will still continue to decline in their lung function and the disease will inexorably progress.
Over the last few years, the important advances in IPF basic, translational and clinical research have revolutionized this field. This is why this series of articles will help experts, specialists, internists, patients, caregivers and policy makers in their decisions and future planning.
Thanks to the open access policy of BMC Medicine, the readers will have access to a wide spectrum of authoritative articles from leaders in this specific field of medicine.
The articles in this series cover some of the most relevant and controversial topics in IPF, thus providing a variegate overview of the current opportunities and future challenges in this emerging area of respiratory medicine.
Highlights from the collection
Reading the Commentary by Pierre-Simon Bellaye and Martin Kolb will help readers understand the complexity of the pathogenesis of this mysterious disease, but also the recent and promising advancements achieved by the expanding community of basic and translational IPF researchers.
Laura Fregonese and Irmgard Eichler, who both work with the European Medicines Agency, describe the failures and the successes in the pathway to approved treatments in IPF.
In their Opinion article, Susan Mathai and her colleagues address the increasingly relevant topic of incorporating genetics into the diagnosis and the treatment of patients with IPF.
Anne-Marie Russell and her colleagues clearly point to the need for putting IPF patients before the condition. Patients, their families and the related not-for-profit organizations, have been making and will keep making major positive contributions to the advancement towards a cure. Kerri Johansson and colleagues have explored the challenge of discussing with IPF patients the causes of their disease to address their expectations and anxieties with regard to their future.
Carlo Vancheri discusses the many (often hidden) links between IPF and cancer, thus highlighting the potential positive pitfalls of considering these two entities as being closely inter-related. In the first of the Research articles in this collection, Simon Walsh and his colleagues explore the complex relationship between pathology and imaging in lung fibrosis.
Finally, a Forum article explores the status of IPF care in a part of the world which is home to the majority of human beings. Four experts, representing the so-called BRIC countries (Brazil, Russia, India and China) have provided for the first time, an authoritative assessment of the potentials and the pitfalls of managing IPF in these areas of the globe.
The future of IPF research is bright. With many new mechanisms of disease being discovered and better analyzed (including genetics) and new promising therapeutic targets on the horizons, there is real hope to better understanding this terrible disease and ultimately reach a cure. After the launch of this collection, BMC Medicine will remain committed to advancing and spreading knowledge about IPF.
Read the full article collection in BMC Medicine:
Idiopathic pulmonary fibrosis: diagnosis, management and new therapies
Guest editor: Luca Richeldi