We talked with Prof. Wim Wuyts about the diagnosis of Interstitial Lung Disease (ILD) and asked him about the pathway to diagnosis, the impact of the time to diagnosis, the disease progression and the challenges to recognize the disease in its early stages.
Prof. Wim Wuyts is associated professor at KU Leuven, Belgium. He is responsible for the unit for Interstitial lung diseases of the department of Respiratory Medicine at UZ Leuven and the pulmonary fibrosis laboratory of respiratory medicine of KU Leuven.
The field of Interstitial lung disease seems to be evolving a lot, could you tell us more about that?
A lot has changed in the field already. 15 years ago there were almost no patients identified and we had no treatment options.
We were treating all our patients with corticosteroids and in the meanwhile we have learned that this kills patients with idiopathic pulmonary fibrosis (IPF). That insight changed the view: we now realize inflammation is not so important in IPF. The development of antifibrotic drugs have changed our world completely. From being a disease with a 15% survival rate at 2.5 years, IPF has become a disease where we can stabilize or at least significantly slow down the reduction in lung function.
On the other hand, we struggled with all the other +200 types of ILD where there was no treatment. We were still treating them with immunosuppressive drugs and were left with the question: is this really helpful, or not? Intensive research found a lot of parallels between IPF and non-IPF fibrotic ILDs and from that the idea came that there were maybe more similarities. Now we have new trials out that show that antifibrotic agents are effective also in patients with all kinds of progressive fibrotic ILDs.
The consequence of that is that we now need to broaden our scope. Instead of looking for the IPF patient, we now need to look for the ILD patient. It is now more then ever important to identify patients that are at risk for some kind of interstitial lung disease.
What is the pathway that an ILD patient typically goes through to come to a diagnosis?
The typical IPF journey is an older patients who becomes short of breath and at first thinks “It is the age”. When it increases he goes to his GP. Often he gets the message “it is your age, don’t bother too much about it” or initiates a puffer because the patient is an ex-smoker and they think about COPD or emphysema. If that is not helpful, often they are then sent to the cardiologist or to the pulmonologist. These local specialists then need to pick up ILD and refer the patient to a specialized center where the differential diagnosis is made.
With the changes in the field, we now also have to focus on picking up the other fibrotic ILDs which are often much younger patients. In the past, we maybe passed the message too much that GPs and local specialists needed to look for IPF patients because that was the most aggressive disease. We underestimated both the amount of patients and the potential for progression of other fibrotic ILDs. We know now more about these diseases and are getting towards better treatment options.
WHy is a timely diagnosis so important? what is the impact on the patient?
You really change the prognosis of the patient. ILD is an irreversible disease and the treatment doesn’t reverse the damage to the lungs. So, when a patient is picked up 6 months later, those 6 months of damage will stay there for ever. If we would pick up 30% of patients more or earlier, that would already be a huge achievement.
The impact is even higher in younger patients of for example 35 years of age. If they remain untreated they may have a survival of 5 years. When we can diagnose and treat them early on we can stabilize them or refer them to lung transplant. That is a tremendous difference for these patients.
How can ArtiQ help in the timely diagnosis of ILD patients?
The ArtiQ technology could help raise awareness. We cannot expect for example GPs to be experts in pulmonary fibrosis, so any support we can give them in raising awareness or putting an alarm that says it might not be a cardiac but a pulmonary problem, will be helpful to get ILD patients referred to their local pulmonologist sooner.
The level of knowledge and experience on ILD with the general pulmonologists varies widely in the world. In Europe, we are blessed that the knowledge of pulmonologists on ILD is very good. There have been quite some investments in training these general pulmonologists in IPF. The ArtiQ software could support by bringing awareness or a sort of alarm also to specialists that are less experienced with ILDs (cardiologists, rheumatologists, maybe even general pulmonologists in some situations).
With the advancements in the field, treatment options are becoming available for more ILD patients. Making sure they are picked up to receive these treatments can make a huge difference for each of these patients.
This infographic on idiopathic pulmonary fibrosis (IPF), the most common type of interstitial lung disease, shows some key numbers on the disease, its diagnosis and the potential of ArtiQ.PFT to assist doctors.