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Pulmonary Fibrosis

Pulmonary fibrosis

09/03/2008

Question:

I was wondering if you could tell me when the first case of pulmonary fibrosis was and how did the disease get its name? I am writing a paper on this disease, because my uncle was diagnosed with it and cant seem to find the information. Thanks for your help.

Answer:

As with many diseases in medicine, pulmonary fibrosis has undergone many definitions and re-definitions over the years as we have gotten better about separating patients into different groups and subsets of disease. In this sense, our definitions have really more evolved rather than the diseases being discovered, per se. 

In 1975, Avril Lebow wrote an article that grouped these conditions into separate diseases based on the appearance of the lung under the microscope. In 1998, Dr. Anna Louise Katzenstein and Dr. Jeffrey Myers published a paper that better defined the different forms of idiopathic interstitial pneumonias into the groups of diseases that we currently use. The most common of these diseases is "usual interstitial pneumonitis" which is often called "idiopathic pulmonary fibrosis".

Over the past 33 years since Dr. Lebow wrote his paper, we have had such a dramatic improvement in our imaging techology, with high resolution chest CT scans, that we are now able to identify patients much earlier in the course of their disease than could be done in the past. Because of the earlier identification of patients with CT, we can perform lung biopsies on patients who are still early in the course of their disease when the diseases can be better separated by their microscopic appearance.

In the past, when patients were usually identified late in the course of their disease, it was not possible to separate patients into different groups of disease based on the microscopic appearance because at end-stage, most of these lung diseases look the same, full of scar and no recognizable normal lung.

For more information:

Go to the Pulmonary Fibrosis health topic, where you can:

Response by:

James N Allen, Jr, MD James N Allen, Jr, MD
Clinical Professor of Pulmonary, Allergy, Critical Care & Sleep Medicine
College of Medicine
The Ohio State University