Even for experts, diagnosing pulmonary fibrosis is a challenge because there are many other interstitial lung diseases that mimic it. Particularly for Idiopathic Pulmonary Fibrosis (IPF), the only way to diagnose it is by considering and ruling out other potential causes. The more you know about what symptoms to look our for and how PF feels, the easier it will be to advocate and get diagnosed as soon as possible. 

How Pulmonary Fibrosis Develops

While the actual cause of pulmonary fibrosis may be hard to pinpoint, certain factors no doubt contribute to its development. These factors can vary from person to person. Pulmonary fibrosis forms in lungs that develop excess scar tissue and continue to create scar tissue.

Normally, the body manufactures scar tissue at sites that experience a sustained injury. In the case of the lungs, the initial injury that triggers the scarring process may be unknown. As scar tissue accumulates, tissues become fibrous. The lungs lose their ability to expand and contract, which makes the exchange of oxygen and carbon dioxide between the alveoli (air sacs) and blood vessels in the lungs and breathing more difficult.

In effect, anything that can potentially damage lung tissue increases the risk of developing PF. Risk factors for developing pulmonary fibrosis include the following:

  • Genetics
  • Cigarette smoking
  • Reflux disease or GERD
  • Environmental exposures to hazardous materials
  • Certain medications
  • Connective tissue disease

4 Reasons Why It’s so Hard to Diagnose Pulmonary Fibrosis

1. A Complicated Diagnostic Process

doctor holding chest x-ray

The body’s inability to take in the needed oxygen supply can set off any number of signs and symptoms. Unless a specialist knows exactly what he or she is looking for, a pulmonary fibrosis diagnosis can be difficult to make. Correctly diagnosing PF requires an integrative diagnostic process that includes a range of specialists such as pulmonologists, radiologists, rheumatologists, pathologists and gastroenterologists, all of whom must have experience with diagnosing fibrotic-based lung diseases.

The complexity of this process, in turn, carries a high probability of misdiagnosis. Results from a 2018 study appearing in the Journal of BMC Pulmonary Medicine shows as high as 55 percent of 600 study participants with idiopathic pulmonary fibrosis reported being misdiagnosed initially. Asthma, pneumonia and bronchitis were the conditions most commonly diagnosed in  place of PF.

2. Delay in Diagnosis

Pulmonary fibrosis typically develops between 50 and 60 years of age. At this age, it’s not uncommon for individuals experiencing symptoms of the disease, such as shortness of breath and fatigue, to attribute them to the effects of aging. Under these conditions, it can be several months before a person sees a physician and yet another several months before a final diagnosis (or misdiagnosis) is made. Additionally, the symptoms are non specific and may be seen in several other conditions.

Diagnostic delays on the doctor’s end can occur for different reasons. In some cases where a definitive diagnosis cannot be made with a high resolution CT scan (HRCT), a tissue sample or biopsy must be taken. If a person is too sick to undergo a biopsy procedure, this can cause delays in the diagnostic process. Also, physicians lacking experience with interstitial lung disease will require more time to make a correct diagnosis, if at all. This may cause delays in referral to a pulmonary specialist. A 2019 study appearing in the Annals of the American Thoracic Society found that doctors and specialists took anywhere from three to five years to reach a diagnosis of PF, based on a sample of 33,780 Medicare beneficiaries.

3. Ruling Out Other Possible Conditions

Idiopathic pulmonary fibrosis, or IPF, is especially hard to diagnose due to the number of interstitial (fibrotic) lung diseases that mimic it. Idiopathic, by its definition, means unknown so all “known” causes must be ruled out first along with other forms of lung disease, including:

  • Connective tissue disease such as rheumatoid arthritis, scleroderma, Sjogrens syndrome that can affect your lungs and cause pulmonary fibrosis
  • Hypersensitivity pneumonitis which happens when dust, mold, bird droppings/feathers or other things that you breathe in irritate your lungs over a period of time and cause scarring
  • Asbestosis which is caused by breathing asbestos fiber used in building materials
  • Sarcoidosis in which there is a growth of tiny collections of inflammatory cells in any part of the body, most commonly the lungs and lymph nodes
  • Drug or radiation treatment-induced fibrosis that can damage your lungs months or sometimes years after the initial treatment

4. Delays in Accessing Specialty Care

doctor in hospital

It should come as no surprise that people who actually have pulmonary fibrosis cannot receive appropriate treatment until an accurate diagnosis is made. In the interim, misdiagnosis of the disease may expose patients to ineffective or even harmful treatments, such as high-dose corticosteroids and bronchodilators. Needed evaluations for lung transplantation may also be put on hold until an accurate diagnosis is made. Consequently, these delays place individuals at risk of suffering from worse outcomes.

A 2011 study published in the American Thoracic Society Journal examined the relationship between delays in access to specialty care and the effects it had on survival time in 129 patients. The criteria for “delay” was defined as the time from when patients experienced bouts of shortness of breath to the time of their first evaluation at a specialty care center. Results from the study showed an average time period of 2.2 years passed before access to specialty care services took place. Researchers also noted a higher mortality rate as a result of delayed access to specialty care.

Ultimately, early detection of pulmonary fibrosis is the key to minimizing harm from the disease itself and from the effects of unnecessary treatments. If you suspect you or someone you know may be showing signs of PF, knowing what symptoms to watch for can make all the difference. Any existing risk factors should also be taken into consideration.

 

To connect with people who have or are impacted by PF:

Join our online forum, and attend our virtual support groups on Zoom.

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5 comments
  • Petty J
    Posted on April 14, 2020 at 2:44 am

    It is important to always consult your doctor if you are disturbed with your health, no matter what. It is better to start curing the illness in the beginning.

    Reply
  • Mary
    Posted on July 15, 2020 at 1:04 am

    I was first diagnosed as having lung nodules in the first cat-scan and when they did a follow-up scan 4 years later they changed it to IPF. This was last year. I was not put in any medication and I’m doing okay so far.

    Reply
    • PF NOW
      Posted on July 21, 2020 at 11:19 pm

      Hi Mary, so glad to know that you are doing okay so far. A lot of people are misdiagnosed or have a delay in diagnosis until they are referred to a specialist. Please visit our website https://pulmonaryfibrosisnow.org/ to learn breathing exercises and other natural ways to control your symptoms and maintain your quality of life.

      Reply
  • Maryann
    Posted on July 24, 2023 at 11:20 am

    I cant get the diagnosis and I want it to take part in clinical trials. My brother has it. Why does this take so long? Then I read where the clinical trials need more people in the early phases of disease. This does not make sense. Could it be that I am on Medicare?

    Reply
    • PF NOW
      Posted on September 18, 2023 at 1:44 am

      Hello Maryann, thank you for reaching out to us. I’m sorry for what you are going through and understand your frustration. Here’s a blog on treatment options for PF and it has a section on how to search on clinicaltrials.gov for a study you may qualify for: https://pulmonaryfibrosisnow.org/2021/10/17/deciding-on-treatment-options-for-pf/
      Participation in a study is not dependent upon what insurance you are on or whether you are on Medicare.

      Reply

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