Early signs of Idiopathic Pulmonary Fibrosis (IPF)

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Video transcript

Ulrich Costabel discusses signs and symptoms of IPF

IPF is a disease that starts actually usually with increased dyspnoea on exertion. The patient experiences dyspnoea first only if he does heavy exercise, let’s say running or at high altitude and if he then is at rest again he is fine. The problem is that as I said these people are elderly and they consider sometimes “Oh it’s normal. I get older so my dyspnoea is due to my age” and also the doctor may feel it. So that is one reason why it’s usually diagnosed late.

Another symptom is cough and these are the two main symptoms: Cough and dyspnoea on exertion.

The clinical signs, and they are quite characteristic for IPF, are the crackles. Fine crackles; they start bibasally and very early in the lateral fields of the lungs, lateral lower fields. There we hear them first and by my experience these are very early signs if you have a true IPF population, almost every patient has this. In the literature it says more than 80%, by my experience more than 95%.

Another sign which you can easily see is the clubbing. If you look at the fingers you see the clubbing, fingers and toes. But that is not so frequent, up 60% have clubbing. It also becomes more prominent if a patient is advanced. Advanced patients all of them have clubbing.

These are the signs and symptoms. So they are mainly non-specific except for the crackles. The crackles are quite impressive and characteristic of IPF, other fibrotic lung diseases may also have crackles, most of them at a lower frequency than in IPF but, let’s say, other lung diseases have not these crackles like pneumonia have a different, COPD, emphysema, cardiac disease with pulmonary congestions have not these type of crackles but different lung sounds. So that is quite unique to IPF and it’s important to educate also doctors to listen to these sounds and to come to the correct suspicion of IPF and take the correct diagnostic measures when they hear these sounds.