Overview

The scar tissue deepens and may make it harder for the lungs to get enough oxygen.

There continues to be no known cure for IPF, but several options for treatment that can help to slow disease progression and enhance a person's quality of life. Appropriate therapy will vary depending on the individual's symptoms, health and the stage of development of the disease.

What is idiopathic Pulmonary Fibrosis?

Idiopathic” means that the cause of Idiopathic Pulmonary Fibrosis remains unknown. Primarily lung disease when there is chronic, irreversible scarring of the tissue in the lungs that envelopes the air sacs. This scarring can make it more and more difficult to breathe over time.

Pulmonary fibrosis, idiopathic is a form of interstitial lung disease. Idiopathic refers to a cause that is not known. Normally tissue around the lungs gets damaged over time, leaving scar tissue in the lungs; this is what happens to people with IPF.

Common symptoms include:

These signs and symptoms are also seen in other lung conditions so more tests are generally undertaken before a diagnosis of IPF is made.

Why is it so important that you treat IPF?

Treatment is crucial as it helps slow down the rate at which the lungs lose function, and allows individuals to stay active for longer.

A few of the drugs, oxygen therapy, pulmonary rehab, healthy lifestyle modifications and regular check-ups that are part of an IPF treatment plan.

Some of the medicines, oxygen therapy, pulmonary rehab, healthy lifestyle changes and regular medical follow-up included in an IPF treatment plan. Therapy may also include the possibility of lung transplant.

Inhaled medicines for IPF treatment

IPF is treated with two fibrotic drugs that are frequently recommended: Pirfenidone and Nintedanib. They have no effect on preventing lung fibrosis from developing but they may help prevent the disease worsening.

Pirfenidone

Pirfenidone is a medicine that is used in adults to treat idiopathic pulmonary fibrosis (IPF) that has an anti-fibrotic action. It does this because it decreases processes in the body which lead to scar tissue building in the lungs.

Medicine is typically introduced at a low levels and slowly raised. This can aid the body to adapt to the treatment.

There could be a few side effects like nausea, stomach discomfort, tiredness, sensitivity to sunlight, and loss of appetite. For these potential side effects, patients are advised to follow the doctor's instructions regarding the medicine and report any unexpected symptoms. Pirfenidone users may be asked to avoid being exposed to extreme amounts of sun.

Nintedanib

This occurs because it interferes with the signals that would otherwise stimulate fibrosis and scar tissue formation.

Nintedanib might help prevent lung function from getting worse in patients with IPF. It is generally given twice a day with food but the amount will always depend on the advice of the doctor.

Side effects are slight diarrhea, nausea, abdominal pain and decreased appetite. Liver function changes may also occur in some patients; in this case, your doctor may order blood tests while you are undergoing treatment.

Nintedanib and Pirfenidone should not be initiated or discontinued or modified without medical advice.
Nerandomilast (Jascayd)
There's a new treatment for IPF called Nerandomilast (Jascayd).

Nerandomilast (Jascayd) is a newer treatment option for adults with IPF. In October 2025, it was approved by the US Food and Drug Administration (FDA).

Nerandomilast is a preferential phosphodiesterase 4B (PDE4B) inhibitor. It is a target for a specific enzyme that plays a role in inflammatory and fibrotic processes. The drug works on these pathways to slow down the progress of lung scarring.

In the FIBRONEER-IPF clinical trial, nerandomilast slowed the decline in lung function compared with placebo during the study.

A critical characteristic of this therapy is that it can be used in combination with an existing antifibrotic agent in some circumstances and according to the prescribing information. This is another way for doctors to treat patients with IPF.

While Nerandomilast is an exciting advance in IPF treatment, it is not a cure. This may vary from country to country and uses may vary by country, please consult a qualified specialist for local treatment options.

Oxygen Therapy

As the disease progresses, the amount of oxygen in the blood (saturation) may start to decrease in some patients. If these occur, oxycanter may be recommended by a doctor.

Some may need extra oxygen to be active or to sleep. Some may need more oxygen than they normally do to be active or to sleep. The proper use of oxygen can assist in allowing patients to participate in everyday activities and can help reduce the shortness of breath experienced by those with low levels of oxygen.

Pulmonary Rehabilitation

It is a program that will be supervised and may consist of exercise, breathing techniques, education, and nutritional advice.It

Many IPF sufferers limit their activity because of their shortness of breath. However, even with no exercise can be a problem of weakness and may make walking even more difficult.

For patients, a pulmonary rehab program can increase their physical endurance, and teach them how to control breathlessness more effectively.

The Lifestyle Changes for People with IPF provide information on lifestyle changes that may benefit those with IPF.

Lifestyle Changes for People with IPF

While medical treatment is essential, there is also a proper daily routine that can help with good health.

Tobacco smoking is discouraged and smoking avoidance is important for individuals who might have been diagnosed with IPF. They should also take care to adhere to their Physician's instructions regarding activities, diets, immunisation, and other diseases.

A well balanced diet can aid to keep the body strong and at a healthy weight. Patients should also be taking good rest and should consult their health care provider in case they have trouble sleeping, or doing any of their normal activities.

It is particularly important not to be picked up because respiratory infections may be more serious in people with chronic lung disease. Depending on the patient's medical history and health, some immunizations might be recommended.

Lung Transplant

A lung transplant can be considered for some with more severe IPF. A lung transplant replaces a diseased lung or lungs with healthy lung(s) donated by someone else.

Physicians take a number of factors into account, such as age, overall health, other disease conditions and the level of lung disease.

If a person might be a candidate for a transplant, the health care team can explain the process of evaluation and the risks involved.

Sometimes, however, the knowledge about the importance of regular monitoring is not utilized.

It's important to perform regular monitoring.

During a doctor's appointment, every day can help a doctor find out if the disease is getting worse and if the treatment is helping.

Any new or deteriorating symptoms such as an increase in dyspnoea, chest pain, fever and sudden onset of cough should also be reported.

When should one seek a doctor’s advice?

Consult a doctor in case of shortness of breath or continuing dry cough. These symptoms can be an indicator that the patient has IPF, but they cannot be ignored.

Conclusion 

IPF-diagnosed patients should speak with their preferred healthcare provider about what is most suitable and safe for them.

Since each patient is unique and has specific needs, it is critical for people living with IPF to consult with a competent health care provider in order to find the most effective and safest treatment.