Pulmonary fibrosis is a progressive, long-term lung disease in which the lungs become scarred, making it difficult to breathe. Though less common than other lung diseases, pulmonary fibrosis can lead to serious complications like pulmonary hypertension, respiratory failure, and lung cancer, so early detection and proactive monitoring of symptoms is critical.

While there is no cure for pulmonary fibrosis, a combination of medications, oxygen therapy, and active lifestyle choices can slow the progression of the disease and reduce discomfort. Read on to learn more about pulmonary fibrosis, including how to recognize and treat pulmonary fibrosis symptoms.

What is pulmonary fibrosis?

Pulmonary fibrosis scars and thickens the connecting tissue in the lungs and alveoli, which are delicate air sacs that transport oxygen into your bloodstream. Over time, the lung tissues grow thicker and it becomes more difficult for the alveoli to deliver oxygen into the body, making it increasingly challenging to breathe. As the disease progresses, you may experience shortness of breath more frequently during everyday activities.

The development of pulmonary fibrosis varies significantly in individuals. For some people, symptoms arise very quickly. For others, they may take years to develop.

Recognizing pulmonary fibrosis symptoms

While the severity of pulmonary fibrosis symptoms vary from one person to another, there are some common symptoms:

• Shortness of breath

• A dry cough

• Fatigue

• Unexplained weight loss

• Aching muscles and joints

• Widening and rounding of the tips of the fingers or toes

Some people with pulmonary fibrosis experience acute flare-ups in which symptoms such as shortness of breath intensify for several days up to several weeks. Medications including antibiotics and corticosteroids, which ease swelling and inflammation, may be used to treat these sudden spikes. Some people may also be placed on a ventilator during these flare-ups to assist with breathing.

Risk factors for pulmonary fibrosis

As with COPD, smoking increases your risk of developing pulmonary fibrosis, but so do other factors like long-term exposure to pollutants and toxins, certain medical conditions and medications, and radiation therapy. Below are the most common risk factors for pulmonary fibrosis.

Age

The aging of the lungs has been linked to higher susceptibility for respiratory diseases. While pulmonary fibrosis can affect infants and children, it primarily occurs in middle-aged and older adults. Most people with the condition develop it between ages 50 and 70.

Smoking

While it’s possible for a non-smoker to develop the condition, pulmonary fibrosis is far more common in smokers. It may occur in people who also have emphysema, a lung disease that falls under the umbrella of COPD.

Professional field

Jobs in mining, farming, and construction often involve long-term exposure to toxins such as silica dust, asbestos fibers, hard metal dusts, coal dust, and bird and animal droppings that damage the lungs. If you work in these fields, you may have a greater risk of developing pulmonary fibrosis.

Gender

While pulmonary fibrosis cases in women have increased in recent years, it continues to affect more men than women overall. This may be due in part to the fact that men are more likely than women to smoke and to work in the professional fields linked to pulmonary fibrosis.

Cancer treatments

Radiation treatments for lung cancer and breast cancer and certain chemotherapy drugs, including methotrexate and cyclophosphamide, may increase your risk of developing pulmonary fibrosis.

The extent of the damage to the lungs during radiation treatments depends on the following factors:

• How much of the lung was exposed to radiation

• Total amount of radiation administered

• If chemotherapy was also used

• Presence of underlying lung diseases

Medication use

In rare cases, other medications have been linked to pulmonary fibrosis, including heart medications like amiodarone, antibiotics like nitrofurantoin and ethambutol, and anti-inflammatory medications like rituximab and sulfasalazine. If you take any of these medications and have concerns, consult with your healthcare provider about potential risks. Be sure to follow your doctor’s recommendations, as abruptly stopping the use of a medication can be dangerous.

Genetics

Researchers believe that there may be a genetic component to certain types of pulmonary fibrosis, as a small percentage of people living with the condition have at least one other affected relative. Research into possible genetic influences is ongoing. If someone in your family has had pulmonary fibrosis, speak with your doctor about your risk. They may refer you to a genetic counselor.

How is pulmonary fibrosis diagnosed?

To determine if you have pulmonary fibrosis, your healthcare provider will ask about your medical history and conduct a physical exam to evaluate symptoms. They may use a stethoscope to listen for abnormal sounds such as crackling when you breathe.

It can be challenging to diagnose pulmonary fibrosis since symptoms overlap with those of other lung diseases. Your doctor may order additional tests, such as blood and imaging tests, pulmonary function tests, and, in some cases, a biopsy to rule out other illnesses and confirm a pulmonary fibrosis diagnosis.

Treating pulmonary fibrosis symptoms

There are a variety of treatment options to alleviate your pulmonary fibrosis symptoms and help you maintain a healthy and active life. Your doctor may recommend one or more of the following common treatments.

Medications

There are two primary medications that slow the progression of lung scarring and preserve lung function: pirfenidone (Esbriet®) and nintedanib (OFEV®). Both medications are similarly effective at treating symptoms and improving lung capacity.

Common side effects of pirfenidone include:

• Nausea

• Vomiting

• Diarrhea

• Heartburn

• Stomach pain

• Headache

• Dizziness

• Pressure or tenderness in the sinuses

• Difficulty falling asleep or staying asleep

• Joint pain

• Weight loss

Common side effects of nintedanib include:

• Nausea

• Vomiting

• Decreased appetite

• Weight loss

Consult with your healthcare provider about the potential risks and benefits of treating pulmonary fibrosis with pirfenidone or nintedanib, and make sure they are aware of any other medications you take.

Oxygen therapy

Oxygen therapy transports more oxygen into your lungs, increasing your tolerance for physical activity and helping you stay active. It can be administered in a hospital during flare-ups or at home. If you have a home oxygen delivery system, your doctor will likely also prescribe a portable device to use when you are on the go. There are different devices for oxygen therapy. Your doctor will recommend one based on several factors including lung function and lifestyle.

Pulmonary rehabilitation

Pulmonary rehabilitation is a program of education and exercise classes, typically in a group setting, aimed at improving lung function and reducing symptoms. In these programs, nurses and respiratory therapists share important information about managing pulmonary fibrosis and teach you how to conserve your energy and increase your level of fitness.

Lung transplants

A lung transplant, in which a damaged lung is replaced with a healthy lung from a donor, is a treatment option for some individuals with pulmonary fibrosis. While it can improve your breathing capacity and tolerance for physical activity, a lung transplant is a major procedure with risks of complications. 

In determining if you are eligible for a lung transplant, your healthcare provider will consider multiple factors such as whether you are in overall good physical health aside from your pulmonary fibrosis and if you have any other life-threatening conditions.

Protect your health by staying proactive

Pulmonary fibrosis can make it difficult to recover from other infections, so it’s important to take steps to boost your immunity and avoid getting sick. In addition to practicing good hygiene and maintaining a healthy distance from anyone who’s sick, stay current on your annual pneumonia and flu vaccinations, and practice healthy habits like staying active, eating well, and avoiding cigarette smoke.

How Alto can support your pulmonary fibrosis treatment

We make it as easy as possible to follow the treatment your doctor has recommended through free hand-delivery of your medication, automatic savings investigations, and easy medication management tools through our website and app. And our team of pharmacists is always here to answer your questions by phone or in-app messaging.

Reach out via phone at 1-800-874-5881 or in-app messaging to learn how Alto can support your pulmonary fibrosis treatment plan.

This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition.

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