As one of the most aggressive forms of lung disease, pulmonary fibrosis (PF) has an average survival time of three to five years after diagnosis. The course of the disease varies in people, and some individuals can live much longer when appropriate treatment is obtained in a timely manner. Unfortunately, PF is difficult to diagnose, causing lengthy delays in receiving treatment. Keep reading to see why pulmonary fibrosis is so hard to diagnose.
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 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:
- Cigarette smoking
- Reflux disease or GERD
- Environmental exposures to hazardous materials
4 Reasons Why It’s so Hard to Diagnose Pulmonary Fibrosis
1. A Complicated Diagnostic Process
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 radiologists, chest physicians and pathologists, 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.
Diagnostic delays on the doctor’s end can occur for different reasons. In order to make a definitive diagnosis, 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
- Hypersensitivity pneumonitis
- Drug or radiation treatment-induced fibrosis
4. Delays in Accessing Specialty Care
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.