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, as well as reduced oxygen in the bloodstream, which can lead to fatigue.

What Are Acute Exacerbations?

An acute exacerbation of IPF is a relatively sudden, unexplained worsening of the condition. Basically, the scarring in a patient’s lungs becomes much worse, and the patient develops extreme difficulty breathing. This shortness or loss of breath is even worse than before. While complications like this can occur for known reasons, there’s no visible cause for an acute exacerbation, such as an infection or heart failure.

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

What Are the Risk Factors?

So far, very little is known about risk factors. Acute exacerbations do not seem to be linked to any of the usual factors, such as:

  • age
  • gender
  • length of illness
  • smoking status
  • previous lung function

Will I Have an Acute Exacerbation?

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 IPF patients 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, around 4 percent.

How Are Acute Exacerbations Treated?

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 are an even less-understood component. There have been no blinded, randomized, or controlled studies aimed at treating acute exacerbations.

Generally, treatment is supportive and/or palliative. The goal is not to reverse the damage, but to help the patient breathe easier and feel better as long as possible. Care may include supplemental oxygen, anxiety medication, and other methods to keep the patient calm and breathing more regularly.

In some cases, drug therapy may be used. If doctors aren’t able to completely rule out an infection causing the exacerbation, then large doses of broad-spectrum antibiotics may be given. If an autoimmune response is suspected, then doctors may give drugs to suppress the immune system, like corticosteroids and other immunosuppressants or even anti-cancer drugs like cyclophosphamide.

What’s on the Horizon?

There’s some hope beginning to surface. New research is emerging, examining several different potential treatments for acute exacerbations of IPF:

  • Fibrogenic mediators are being studied for their effects on slowing the formation of scar tissue.
  • Fibroblast proliferation, a normal bodily process involved in wound healing, is being examined.
  • New and different immunosuppressant drugs and antibiotics are being tested for their potential benefits.
  • The removal of certain immune system cells is being looked at 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.