A diagnosis of idiopathic pulmonary fibrosis (IPF) brings significant life changes. For many, the first sign is a persistent dry cough or feeling breathless during simple activities, like walking to the mailbox or climbing a flight of stairs. As the condition progresses, this shortness of breath can affect daily independence and energy levels. While the term “idiopathic” means the specific cause is unknown, the impact is very real: the lungs become scarred and stiff, making it harder to breathe deeply and transfer oxygen into the bloodstream.

Treatment is critical because IPF is a progressive disease. While current medical therapies cannot reverse existing scarring, they play a vital role in slowing down the damage to healthy lung tissue. The goal is to preserve lung function for as long as possible and improve quality of life. Because the rate of progression varies significantly from person to person, treatment plans are carefully tailored. Decisions often depend on how advanced the scarring is, the patient’s tolerance for medication side effects, and overall health status (National Heart, Lung, and Blood Institute, 2023).

Overview of treatment options for Idiopathic Pulmonary Fibrosis

The primary objective of treating IPF is to slow the rate of lung function decline. In the past, treatments focused on suppressing the immune system, but medical understanding has shifted. Today, the standard of care centers on antifibrotic therapies that directly target the scarring process.

In addition to medication, comprehensive care often includes pulmonary rehabilitation to build endurance and supplemental oxygen therapy to ensure the body gets enough oxygen during activity and sleep. For qualified candidates with advanced disease, lung transplantation remains a crucial consideration. However, for the majority of patients, consistent medication therapy is the daily method for managing the condition and extending life expectancy.

Medications used for Idiopathic Pulmonary Fibrosis

There are currently two specific antifibrotic drugs approved for treating IPF: nintedanib and pirfenidone. These are considered the first-line standard of care. Clinical studies show that both medications effectively slow the decline in forced vital capacity (FVC), a key measure of lung function, by roughly 50% per year compared to placebo.

Nintedanib is a tyrosine kinase inhibitor. It is typically taken as a capsule twice daily. Pirfenidone is an antifibrotic agent taken as a tablet or capsule, usually three times a day with food. Choosing between them is often a matter of managing side effects and patient preference, as they are similarly effective.

Because gastrointestinal reflux disease (GERD) is highly prevalent in patients with IPF and may contribute to lung injury, doctors frequently prescribe proton pump inhibitors (PPIs) like omeprazole or pantoprazole. Additionally, medications may be used to manage a persistent cough, which can be physically exhausting. It is important for patients to understand that antifibrotics are maintenance drugs; they do not provide immediate symptom relief like an inhaler, but work silently to protect the lungs over time (Pulmonary Fibrosis Foundation, 2024).

How these medications work

Antifibrotic medications interrupt the biological pathways leading to scar tissue. In Idiopathic Pulmonary Fibrosis (IPF), the body’s repair process overproduces fibrous tissue, stiffening the lungs. Nintedanib blocks specific enzymes (growth factor receptors) that signal fibroblast cells to multiply and create scar tissue, limiting thick tissue buildup.  Pirfenidone works similarly but distinctly, reducing collagen and other fibrosis/inflammation-contributing substances. These drugs keep lung tissue softer and more distinct, aiding easier lung expansion for a longer period.

Side effects and safety considerations

Both nintedanib and pirfenidone commonly cause gastrointestinal side effects (nausea, diarrhea, loss of appetite), often managed by taking medication with food or adjusting the dose. Weight loss is also possible and requires monitoring.

Pirfenidone specifically causes photosensitivity, necessitating high-SPF sunscreen and protective clothing to prevent severe sunburn/rash. Both drugs require regular liver function blood tests and are generally contraindicated in severe liver disease or pregnancy. Patients should seek medical attention for jaundice, severe abdominal pain, or uncontrollable diarrhea.

Since everyone’s experience with the condition and its treatments can vary, working closely with a qualified healthcare provider helps ensure safe and effective care.

References

1. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov
2. Pulmonary Fibrosis Foundation. https://www.pulmonaryfibrosis.org
3. Mayo Clinic. https://www.mayoclinic.org
4. American Lung Association. https://www.lung.org