Idiopathic pulmonary fibrosis (IPF) is a lung condition affecting more than 130,000 Americans. Still, IPF is a relatively unknown condition.

IPF causes tissue inside the lungs to scar and thicken. This scarring worsens over times. The damaged lung tissue can’t take in oxygen as efficiently, making it difficult to breathe. The term idiopathic means that, unlike some cases of pulmonary fibrosis, doctors aren’t able to pinpoint its exact cause.

Possible Causes

Depending on your medical history and lifestyle, there are many possible causes of IPF. In most cases, doctors don’t know exactly what causes pulmonary fibrosis. It’s likely caused by a combination of environmental and genetic factors.

Pollution and Toxins

Exposure to environmental, household, and occupational pollution is thought to contribute to IPF. The likelihood is higher with prolonged and long-term exposure. Some likely causes are:

  • asbestos
  • dust from silica and hard metals
  • bird droppings
  • household dust
  • grain and wood dust
  • dust, droppings, and particles from cattle and other types of farming
  • toxic fumes

Radiation Treatment

Radiation therapy near the chest, like that used to treat breast and lung cancers, can lead to scar tissue in the lung. The severity of the pulmonary fibrosis generally depends on how much of the lung was exposed to radiation, the length of treatment, and the level of radiation.

Prior or Existing Illness

Viral infections may be possible causes of IPF, such as:

There are other medical conditions that are associated with pulmonary fibrosis, such as tuberculosis, rheumatoid arthritis, and lupus. Gastroesophageal reflux disease (GERD) may also play a role. It’s thought that those who suffer from GERD may inhale droplets of stomach content into the lungs, leading to the formation of scar tissue.

Medications

Several medications are also believed to be associated with IPF. Some of these are the chemotherapy drugs methotrexate, bleomycin, and cyclophosphamide. Some heart medications may also be to blame, like the beta blocker propranolol and arrhythmia drug amiodarone. Another class of drugs studied for its role in idiopathic pulmonary fibrosis is antibiotics, particularly sulfasalazine and nitrofurantoin.

Genetic Factors

Little is known about the possible genetic component of IPF. While there seems to be a hereditary link or connection, more research is needed in this area.

Symptoms

The main symptom of IPF is shortness of breath. This comes from the lungs being unable to efficiently bring in oxygen and pass it into the bloodstream. Other symptoms include:

  • fatigue
  • weight loss not related to changes in diet or exercise
  • dry cough that persists for 30 days or more
  • a crackling sound in the lungs
  • muscle and joint aches and pain
  • enlarged, rounded fingertips and nails, known as clubbing

Risk Factors

Certain factors can increase your risk for developing IPF. These include:

  • age (between 40 and 70)
  • male gender
  • smoking cigarettes (previous or current)
  • working around pollution like dust or fumes
  • family history of IPF

While IPF is still poorly understood by both the general public and the medical community, there are a few organizations leading the fight for more research to search for a cure. As awareness is raised, and research advances, there’s hope that there will soon be greater knowledge of where this condition comes from, and how to combat it.

 

Read Video Transcript »

Idiopathic Pulmonary Fibrosis is a progressive stiffening of the lungs. Now the lung is meant to expand and contract, like a balloon. And it takes in oxygen to help diffuse into the blood stream to get to our tissues. And it's made up of a tissue call fibrous cells and they give the lung its structure. However, with Idiopathic Pulmonary Fibrosis, there's an over production of these fibrous cells and the lung becomes stiff and can't expand and take in as much oxygen as it normally does.

Causes

So the exact causes of Idiopathic Pulmonary Fibrosis are unknown. However we do have a fairly good idea of what some possible causes could be and they come from chronic trauma to the lungs that causes damage. Some fo these things could be smoking, which causes chronic irritation to the lungs. Another thing could be dust from a worksite. Or even the effects of heartburn which causes acid to come up from the esophagus and go back into the lungs.

Symptoms

The usual symptoms of IPF would be anything that affects the lungs as you can imagine. If you can't breathe very well, you'll be short of breath. Now in the beginning of the disease, you may be able to walk several blocks without any problem. But as it progresses, it's hard to walk one block without having to sit down because you're so short of breath. Another very common symptom is a chronic cough. It may or may not be productive.

Treatments

The treatment for IPF used to not be very good. We tried anti-inflammatory medications and steroids, but they never really worked. Because, as we know now, the disease is not about inflammation but more about the proliferation of these fibrous cells. However, there are 2-new medications that actually target the fibrous cells and these two medications are Esbriet and Ofev. What they do is that they block the proliferation of these fibrous cells so it really slows the progression of the disease.

Exacerbations

An exacerbation is something that causes a worsening of the symptoms. And an exacerbation would come from something like an infection. The infections in someone with Idiopathic Pulmonary Fibrosis can be very dangerous and so we treat them right away with antibiotics and steroids to stop the inflammation from the infection, not from the disease itself. Other things would be supplemental oxygen so you see people wearing oxygen tanks. This can help as well.

Living with IPF

Lifestyle changes that people should consider if they do have Idiopathic Pulmonary Fibrosis would be: one, stopping smoking, two, treating their heartburn, and three, doing something called pulmonary rehab which are exercises designed to improve your lung function. Although in the past, we never had good treatment for Idiopathic Pulmonary Fibrosis, now with these new medications we can really preserve the quality of life. So make sure to take those medications regularly, do your pulmonary rehab, and always consult regularly with your physician.