What is IPF?

Idiopathic Pulmonary Fibrosis = IPF. IPF roughly means “lung scarring of an unknown cause” (but this is a diagnosis in itself)

It is only one possible diagnosis in the field of interstitial lung diseases. Most people who search for information about “pulmonary fibrosis” online come across information specifically about IPF. Not all pulmonary fibrosis is IPF !! The information that you find can be scary and may actually not apply to other cases of pulmonary fibrosis. I highly recommend checking with your doctor whether the information that you found online actually applies in your case.

Further details about IPF:

  1. IPF is a diagnosis of exclusion. Most patients I see find this concept very hard to understand. IPF is not something we “measure”. Basically, when scarring on the lungs is found on chest CT imaging, your doctor will try to find a cause for it (occupational exposures, autoimmune conditions, certain medication, sensitization to things that we inhale from the environment etc.). When no obvious cause is found, and the chest CT scan pattern of scarring is compatible, we call this IPF (idiopathic pulmonary fibrosis). As you can imagine, we can only rule out a limited number of causes. This means that…
  2. Not all cases of IPF behave in the same way. Some patients are stable for a very long time, while others can progress more rapidly.
  3. However, IPF is generally expected to gradually worsen over time (at varying rates from person to person).
  4. Antifibrotic treatment for IPF exists. Antifibrotic (or “anti-scarring”) medications are not expected to improve IPF, but can slow down the worsening of the disease. Therefore, there is a chance to stabilize the scarring process if going on antifibrotic medication.
  5. Two antifibrotic medications for IPF are approved (as of 2023) – nintedanib and pirfenidone. They are both expected to roughly half the rate of lung function decline.
  6. Nintedanib and pirfenidone can have some side-effects, but these are generally manageable. The side effects are different for nintedanib vs. pirfenidone and patients are involved in the choice of one drug versus the other (depending on personal circumstances).
  7. There are a lot of clinical trials to research new medications for IPF. Going on a clinical trial can be a positive experience for patients, with regular clinical follow-up as part of the trial and the chance to potentially have access to the most recent medication available. Clinical trials are not for everyone, but I do recommend checking with your doctor if there are any trials available in your area. Going on a clinical trial does not automatically mean that you would get a better health outcome, but patient participation is the only way to determine which drugs are the most effective for the treatment of IPF, and the safest.