Pulmonary Fibrosis: Diagnosis and Treatments

You may have heard of chronic obstructive pulmonary disease (COPD), a progressive lung disease that affects lung function, but what about pulmonary fibrosis?

Pulmonary fibrosis is another difficult disease whose progressive, lifelong diagnosis also affects lung function. However, pulmonary fibrosis is rarer and can sometimes be more severe than COPD.

The condition involves damage to the lung tissue such that it scars, which leads to shortness of breath. As scarring thickens, symptoms progressively worsen, which often leads to more breathlessness and a further decline in lung health.

Today, Carda is here to talk about pulmonary fibrosis, including diagnosis techniques and treatment options. Read on to learn more about supporting pulmonary recovery and overall wellness with Carda.

What Is Pulmonary Fibrosis?

Pulmonary fibrosis happens when lung tissue incurs damage and scarring. The scarring is inflammatory, which thickens the tissue and makes the lung stiff (in turn making it hard to breathe).

The lung tissue that pulmonary fibrosis damages is called interstitial tissue, which is why this medical condition falls under the interstitial lung disease classification.

Interstitial tissue is the connective tissue between blood vessels, alveoli (air sacs), and other inner pulmonary parts. The alveoli are already vulnerable structures due to their thin cell walls.

Scarring makes the air sacs even more vulnerable — thickening alveoli walls have more trouble expanding, which in turn makes it harder for them to exchange oxygen into the affected patient's bloodstream.

As this tissue progressively scars and thickens, patients will experience more and more breathlessness, among other symptoms. Other symptoms include:

  • Persistent dry cough
  • Fatigue
  • Unexplained weight loss

The progressive timeline for pulmonary fibrosis varies — some patients may experience rapidly worsening symptom progression, while other patients might experience mild symptoms for years before a more severe onset presents.

What Is Idiopathic Pulmonary Fibrosis (IPF)?

The term idiopathic refers to an unknown pathology or disease origin. Idiopathic pulmonary fibrosis (IPF) occurs when the causes of scarring and lung tissue damage are unknown.

Although the specific causes for interstitial lung tissue scarring often remain unknown, certain risk factors make a patient more likely to develop this lung condition. Smoking is a risk factor for pulmonary fibrosis, as are genetics — this lung disease can run in families.

However, most of the time, the causal risk factors remain unidentified, and the disease is idiopathic in nature.

How Is Pulmonary Fibrosis Diagnosed?

A provider can diagnose pulmonary fibrosis through several different means, including pulmonary function tests, imaging, lung tissue samples, or blood tests. Pulmonary function tests are a good indicator as to how much trouble the patient experiences with breathlessness, as is how rapidly the progression of the disease occurs.

If your patient demonstrates a concerning score on their pulmonary function test, or if you have other cause to believe they might have pulmonary fibrosis (such as medical and family history), then it may be time to pursue a pulmonary fibrosis diagnosis.

Let's take a more in-depth look below at how this lifelong disease can be diagnosed.


Two imaging tests, a chest X-ray and a computed tomography (CT) scan can show lung tissue scarring and help a provider to diagnose pulmonary fibrosis.

A chest X-ray can visually encapsulate pulmonary interstitial scar tissue and help track changes in the amount of scar tissue growth.

However, sometimes scarring does not show up on a chest X-ray. In this case, you may want to order a computed tomography scan. CT scans produce cross-sectional images and can help depict any scar tissue present, even if it does not show on an X-ray.

Lung Tissue Samples

Imaging through a chest X-ray or CT scan is a good first step toward ruling out pulmonary fibrosis. However, sometimes even a CT scan can fail to provide evidence for a diagnosis, in which case lung tissue samples would be the next step in the testing process.

A lung biopsy is a medical procedure that removes a tiny quantity of lung tissue for further laboratory analysis. There are two different ways to acquire lung tissue for laboratory purposes: a bronchoscopy or surgery.

In a bronchoscopy, a provider uses a bronchoscope, which is a thin and tiny flexible tube, to remove extremely small amounts of lung tissue. The bronchoscope reaches the patient's lungs through the mouth and nose and down into the respiratory tract.

A bronchoscopy does not involve surgical incisions and is, therefore, usually a more favorable way to obtain a lung tissue sample. However, this procedure only yields trace amounts of sample, and sometimes the minuscule quantity is not enough to determine whether a patient has pulmonary fibrosis.

Surgery is the best way to ensure a large tissue sample that can help rule out a pulmonary fibrosis diagnosis. A surgical biopsy can still be minimally invasive through a VATS procedure (video-assisted thoracoscopic surgery) or alternatively through a more involved and invasive surgery that opens the chest cavity (a thoracotomy).

Blood Tests

Blood tests can also help determine a pulmonary fibrosis diagnosis — they can test for other conditions to help rule them out.

Additionally, blood tests can help providers evaluate a patient's liver and kidney function, as declines in either of these organs' functioning may be indicative of a pulmonary fibrosis diagnosis.

Can the Lungs Recover From Pulmonary Fibrosis?

Unfortunately, the lungs cannot recover from pulmonary fibrosis — this is a lifelong condition that progresses over time. There is no way to reverse the tissue damage or lessen the scarring; ultimately, a patient might need palliative care as they deal with the more severe end of their symptoms’ progression.

While there is no cure for pulmonary fibrosis, there are treatment options that can help slow disease progression.

How Is Pulmonary Fibrosis Treated?

Treatment of idiopathic pulmonary fibrosis involves interventions that can help to slow disease progression and improve patient quality of life. Let's explore some of these treatment options below.


There are two different medications — Pirfenidone (Esbriet) and Nintedanib (Ofev) — that can help decrease the pace at which lung tissue scarring occurs. These two medications are FDA-approved, and their ability to help slow lung scarring can, in turn, help protect a patient's lung function for a longer time to stave off disease progression.

Often, a provider will co-prescribe gastroesophageal reflux disease (GERD) medication alongside pulmonary fibrosis medication because there is a correlation such that many patients who have pulmonary fibrosis also experience GERD.


If pulmonary fibrosis rapidly progresses and severely affects a patient’s quality of life, a lung transplant may be the best intervention course.

A lung transplant is not for every patient, though, as this procedure has risks, including infection or tissue rejection. Patients should discuss this option in detail with their pulmonologist first.

Oxygen Support

Supplemental oxygen can help improve a patient’s quality of life because it can help alleviate breathlessness.

When a patient experiences shortness of breath less often, their abilities to perform day-to-day tasks, experience a good night’s sleep, and have a general sense of wellness all increase.

Pulmonary Rehab

Pulmonary fibrosis can cause difficulties breathing, which can, in turn, affect a patient’s lung and heart function due to diminished oxygen and blood flow.

A pulmonary and cardiac rehabilitation program is another treatment option to help relieve symptoms of pulmonary fibrosis. Pulmonary rehab can also help foster awareness and management practices for the condition's progression.

Rehabilitation aims to improve a patient’s cardiovascular and pulmonary health through monitored physical activity, dietary guidance, education, and support groups. These interventions may help facilitate better heart and lung health and improve overall patient wellness.

If you think your patient could benefit from a pulmonary rehab referral, Carda is here to help. Carda offers at-home personalized virtual rehabilitation programs that have many benefits over traditional in-person rehab facilities, including easier, cheaper, and more effective at-home care.

The Bottom Line

Pulmonary fibrosis is a serious lung disease whose lifelong progression has no cure. With the condition, a patient experiences damage to the lung tissue such that it scars and thickens. Scarring can yield symptoms such as shortness of breath, breathlessness, fatigue, and dry coughing.

While there is no way to reverse the damage to the lung tissue, there are treatment options that can delay the decline in lung health.

If your patient suffers from pulmonary fibrosis, medication, surgery, oxygen therapy, or pulmonary rehabilitation could be a life-altering change that increases their lifespan and improves their overall quality of life. Get started today with Carda to help support pulmonary wellness.


How Is Pulmonary Fibrosis Treated? | American Lung Association

Diagnosis | Pulmonary Fibrosis Foundation

Idiopathic Pulmonary Fibrosis | Treatment | NHLBI, NIH

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