RESEARCH AT CPC-M - Rare disease: Idiopathic pulmonary fibrosis


Rare disease: Idiopathic pulmonary fibrosis

How could the constant deterioration of the disease be slowed down?
Is the lung vitality capacity an indicator?

Although idiopathic pulmonary fibrosis (IPF) is rare, it is not curable. IPF is a lung disease of unknown etiology that is characterized by progressive scarring of the pulmonary parenchyma that leads to decreased lung function capacity and respiratory failure in the course of time. This course of disease might individually occur slower or faster in some patients and up to now it is not predictable how the disease will develop in a single patient. The median term of survival after diagnosis is only about 3-4 years. For a certain subgroup of patients undergoing lung transplantation is the last possible option.

What is known so far: IPF patients often undergo acute, significant deterioration of the disease, so-called exacerbations, accompanied with sudden, increased respiratory distress. However, exacerbations significantly increase the risk of death: most IPF patients die within the following 12 months after such exacerbations.

Grafic illustration of lung fibrosis
Illustration lung fibrosis
© Angelika Kramer

A main issue is therefore: could it improve the life expectancy of IPF patients if exacerbations are detected earlier?

The pilot study AE-IPF aims to answer this question. The study is conducted at four DZL locations, including the DZL Munich site, the CPC-M. The key question: If the patients regularly measure the vital capacity of the lung at home and forward the results to a central location - would it then be possible for the physicians to recognize an exacerbation in time? And would that have a positive effect on the disease progress?


Vital capacity (VC) – possible value for the early detection of exacerbations?

The vital capacity (VC) indicates how much volume or how much air the lung can breathe in and out. If this value deteriorates, an accelerated progression of the disease can be expected. In addition, unpredictable exacerbations are increasing, that on the other hand significantly deteriorate the disease - the mortality risk increases. A vicious circle.

IPF patient with spirometry
Study patient with spirometry device and App

Therefore we provide the participants of the AE-IPF pilot study with adequate technology to perform the measurement of all the relevant lung function parameters themselves at home and to transmit the values to the physicians:


  • A device for spirometry, the measurement of the vital capacity of the lung
  • A tablet including the app "My Health Monitor"

The patients then perform measurement of their vital capacity every morning for one year with the spirometry device which then automatically transmits data via Bluetooth to the tablet that then stores all measurement results in an electronical diary. The responsible physicians as well as the study physicians can read the results at any time. In addition, the study participants are provided with a diary and answer a questionnaire on their quality of life every 12 weeks.

The final goal of the researchers: to detect and treat exacerbations faster - which could slow the progression of the disease.

IPF - Higher survival with antifibrotic therapy

Does antifibrotic therapy for idiopathic pulmonary fibrosis (IPF) have an impact on mortality? As there is a paucity of observational data, the researchers around Prof. Jürgen Behr cand Dr. Marion Frankenberger looked at the database of the INSIGHTS-IPF registry. They analysed data from a cohort study of IPF patients from 20 interstitial lung disease expert centres in Germany.
The results showed that 1-year and 2-year survival rates were significantly higher for patients with antifibrotic therapy - The risk of death was reduced by 37%. The results were robust (and remained statistically significant) on multivariable analysis and were published in European Respiratory Journal (see Link to Publication on the right).