When Scleroderma Affects the Lungs: Why it’s Important to Act Early.

Dr. Bruni, nowadays, we, uh, spoke more and more about VEDOS, a very early diagnosis of systemic sclerosis.

Why is important to have an early diagnosis?

So thank you, Ilaria.

That's a very relevant point.

The diagnosis of the disease sometime comes very early, meaning few months or few, uh, weeks even sometimes after the very big first manifestation or first symptoms of the disease itself, while sometimes it takes years before the condition and the overall body status gets a name.

And this is very relevant because once systemic sclerosis is there present as a condition and has caused damage, it's very hard, almost impossible, to get the damage back, so to remove tissue which has been damaged by the disease at a skin level, at a joint level, at lung level.

So the earlier we are able to detect the condition, the systemic sclerosis itself, the better, because at least we are able to start monitoring the patient and screening the patient for conditions which have not manifest yet or which are not even there on images.

So we would be able to see the patient in his very baseline status when the things are as clean as possible, and that's the best starting point we can have.

We do not has- have as of now many way of preventing the disease evolution, of avoiding the appearance of certain complications.

Let's say primary prevention, this is what it's called.

But we do have the chance to act early on the conditions, on the different organs and the different manifestations the disease can present with our current medical weapons, let's say our medical possibilities.

So the earlier we get the diagnosis, the earlier we can start monitoring, screen the patient, and doing our regular checkups, the earlier we can start the treatment.

And this allows at least avoiding or delaying the damage that the disease usually brings.

So why it is important to identify the progressive element of ILD rather than waiting for more significant elements?

Yeah, that's a very relevant, practice relevant, and very also patient relevant question.

Because we know overall that if you have certain complications or manifestations of the disease as interstitial lung disease is, there is higher risk of having more complications later on in your life.

And interstitial lung disease is typically one of those complications that tend to change over time and more frequently getting worse than getting better.

We do have knowledge accumulated over time from other diseases having lung complications similar to the, uh, SSC ILD, in which there is tendency to keep on losing the way the lung function and the way then, as a consequence, the patient functions in their daily life.

We should be aware that as the status of medical possibility and medical care is today, once the scars in the lung, in the lungs are there, we do not have that many chance for the lung tissue itself to go back to the way it used to be.

And that's the same when you do have a bad accident, for example, and that turns up with a big scar on your body.

Yes, okay, we do have aesthetic surgery for that now.

We do not have that in the lungs.

So we cannot replace that little part of scarred lung unless you get a full, uh, lung transplantation, and we do not have the chance with our medications, the way we use them now and what we have available now, for the tissue to go back to normal.

And especially if you tend to be a patient who worsens rapidly in a short period of time, usually in a year time or in a couple of years' time, then your chance of having further worsening over time is even higher.

And this further worsening has been associated in many conditions as well as in, um, in, in systemic sclerosis with higher mortality.