Diagnosis and treatment of idiopathic pulmonary fibrosis 1

Published on October 6, 2015   27 min

Other Talks in the Series: Topical Talks

0:00
Hello. My name is Luca Richeldi. I'm a professor of respiratory medicine at the University Hospital of Southampton in the United Kingdom. And today, I will speak about the diagnosis and treatment of idiopathic pulmonary fibrosis.
0:16
So before going into the specifics and the details of pulmonary fibrosis, let me review with you this recent article published in the New England Journal of Medicine. And discussing the topic of fibrosis, which as you see is defined as a common pathway to organ injury and failure. And there are at least three concepts that I would like to underscore with you from this paper. The first one is that the mechanism of fibrosis and the resultant organ failure account for at least one-third of deaths worldwide. And this has not been recognized properly before, and that means that fibrosis together with other cause of disease is a major driver in mortality in every country in the world. The second concept is that fibrosis having adverse effects, basically in all organs, is a very attractive therapeutic target, and even more so because, and it is the third concept I would like to discuss with you, is that contrary to the widely held perception that scar is permanent, we know now from animal models and from preclinical data that there is evidence pointing to the fact that organ fibrosis is highly plastic in nature. That means that the idea of potentially revert fibrosis is something that needs to be considered as an achievable target for any therapeutical trial.
1:45
This is true for almost every organ in the body. And you see that the eye, the skin, the liver, the heart, the lung, the pancreas, and the kidney, they're all affected by fibrotic conditions and they all have consequences that manifest in different disorders in these organs.
2:10
The good news are that we are starting to see some way to treat these organ failures due to fibrosis, and you see a few of them like Rho pathway that are currently under investigation with target drugs to treat failure in different organs and these include TGF-beta receptors, integrins, various growth factors, cytokines, vasoactive peptides, and the biosynthesis of extracellular matrix. Now this means that there have been many attempts so far to treat fibrosis in many organs.
2:46
However, in particular for the lung, these attempts have been particularly unsuccessful. This is the cover of a recent book that has been published on orphan lung diseases, that is also known as interstitial lung diseases. And this is a group of diseases characterized by fibrosis as the major mechanism of disease, and they're also referred to as rare lung diseases, because taken individually, some of them are extremely rare and some of them, they are less rare. However, the important message here is that when you lump together all of these so-called orphan lung diseases, they account for about one-third of all lung disorders. This means that interstitial lung diseases represent a relatively large number of diseases that can affect the lung and they represent also a mathematical need so far.
3:45
So the classification of interstitial lung disease has been attempted since many years ago. Actually, one of the first classification has been published by the American Thoracic Society and the European Thoracic Society in 2002, and has been recently revised in 2014. So we have at least five major categories of interstitial lung disease. The first one being interstitial lung disease related to exposure. This exposure can be occupational, it can be environmental, it can be avocational, or it can be exposure to drugs, and many drugs can cause interstitial lung disease. So this first group is interstitial lung disease with a known cause and an exposure, mainly. A second group is interstitial lung disease associated with connective tissue disorders, mainly scleroderma, rheumatoid, sjögren, but basically any connective tissue disease can affect the lung in the form of interstitial lung disease including polymyositis, dermatomyositis. And very often in patients with these connective tissue diseases, the lung involvement represents the major cause of mortality. The third group is represented by granulomatous disorders of unknown origin, and the prototype of these disorders is sarcoid. A fifth group is represented by a mix of different disorders and these disorders, usually, they have a relatively low prevalence and frequency in the whole population, but they represent huge challenges, both for diagnosis and for treatment. I'm just mentioning vasculitis of the lung, Langerhans' cell histiocytosis, eosinophillic pneumonia, neurofibromatosis, and lymphangioleiomyomatosis, just to name a few. Now today, we will focus on another group, which is a group of idiopathic interstitial pneumonia, so these are interstitial lung diseases with no known cause, and no disease associated. So these diseases, they affect primarily and exclusively the lung and they don't have any known cause.
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Diagnosis and treatment of idiopathic pulmonary fibrosis 1

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