What is idiopathic pulmonary fibrosis (IPF)?

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that features the formation of scar tissue between the walls of the lungs’ air sacs. As this scar tissue thickens and stiffens, the lungs aren’t able to take in oxygen as efficiently.

IPF is progressive, which means that the scarring worsens over time.

The main symptom is shortness of breath. It also causes reduced oxygen in the bloodstream, which can lead to fatigue.

An acute exacerbation of IPF is a relatively sudden, unexplained worsening of the condition. Basically, the scarring in a person’s lungs becomes much worse, and the person develops extreme difficulty breathing. This shortness or loss of breath is even worse than before.

A person with exacerbations may have medical conditions, such as an infection or heart failure. However, these other conditions won’t be severe enough to explain their extreme breathing problems.

Unlike exacerbations in other lung diseases such as chronic obstructive pulmonary disease (COPD), in IPF it’s not simply a matter of having extra trouble breathing. The damage caused by IPF is permanent. The term “acute” simply means that the deterioration happens rather quickly, typically within 30 days.

So far, very little is known about risk factors for IPF exacerbations.

Acute exacerbations for IPF don’t seem to be linked to any of the usual risk factors for lung disease exacerbations. These include:

Without understanding the risk factors, knowing if you’ll have an acute exacerbation is difficult to predict. Researchers don’t necessarily agree on the rates of acute exacerbations.

One study determined that about 14 percent of people with IPF would experience an acute exacerbation within a year of diagnosis and about 21 percent within three years. In clinical trials, the incidence seems to be much lower.

There’s little in the way of effective treatment for an acute exacerbation.

IPF is a poorly understood condition within the medical field, acute exacerbations even more so. There have been no blinded, randomized, or controlled studies aimed at treating acute exacerbations.

Generally, treatment is supportive or palliative. The goal isn’t to reverse the damage, but to help the person breathe easier and feel better as long as possible.

Care may include supplemental oxygen, anxiety medication, and other methods to keep the person calm and breathing more regularly.

Drug therapy

In some cases, drug therapy may be used.

Currently, two medications have been approved by the U.S. Food and Drug Administration (FDA) to treat IPF:

  • nintedanib (Ofev), an antifibrotic medication
  • pirfenidone (Esbriet, Pirfenex, Pirespa), an antifibrotic and anti-inflammatory medication

If doctors aren’t able to completely rule out an infection causing the exacerbation, they may recommend large doses of broad-spectrum antibiotics.

If an autoimmune response is suspected, doctors may prescribe drugs to suppress the immune system. These may include corticosteroids, other immunosuppressants, or even anticancer drugs such as cyclophosphamide.

Promising research is emerging that examines several potential treatments for acute exacerbations of IPF:

  • fibrogenic mediators and their effects on slowing the formation of scar tissue
  • fibroblast proliferation, a normal bodily process involved in wound healing
  • new and different immunosuppressant drugs and antibiotics
  • the removal of certain immune system cells to see how this may slow the progression of IPF or reduce the risk of acute exacerbation

While it’s much too soon to know whether any of this research will result in an effective treatment for acute exacerbations, it’s encouraging to know that more attention is being paid to this relatively unknown condition. Learn more about the future of IPF treatment here.