Idiopathic Pulmonary Fibrosis (IPF) Prevalence and incidence

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Video transcript


Ulrich Costabel discusses the prevalence and incidence of IPF

IPF is a rare disease. A rare disease by definition is a disease that affects less than 1 of 2.000 people. That means around 50 per 100.000 and the prevalence of IPF is if you define it narrowly, don’t include any unclear cases, don’t include all the IIP’s definitely below 50 per 100.000. So the estimates are somewhere if you define it narrowly between 20 and 30 per 100.000.

The incidence of IPF if you define it narrowly is around 7 to 10 per 100.000 people.

Well, a rare disease is not the same as an orphan disease. Frequently you hear also a disease is an orphan disease. What is the difference between orphan and rare? Orphan is a disease where you don’t know much about it, where the patient is lost because there is no public awareness, nobody knows what causes the disease, nobody knows how to treat the disease. If you look at this and look at IPF in most part I think until now IPF is really an orphan disease, not only a rare disease because we don’t know what causes it. For instance, in Germany just last year the first IPF patient support group came up. Before there was no patient support group at all.

What is the reason actually why we don’t have so active patient support groups like for cystic fibrosis? Also a rare disease. Or for lymphangiomyomatosis (LAM). It’s a disease only affecting women, a very rare disease. Much rarer than IPF but this rare disease is not an orphan disease anymore, LAM. We know the cause, it’s a mutation in the traverse sclerosis gene, we have now effective treatment more or less, Sirolimus. We know a lot about the pathogenesis and have very strong support groups. So LAM, much rarer, cannot considered actually anymore an orphan disease by my opinion whereas IPF is still an orphan disease and I think we have to improve that in the future and probably will also happen. So then IPF will still be a rare disease but not orphan anymore.