How Is Idiopathic Pulmonary Fibrosis Treated?

Idiopathic pulmonary fibrosis (IPF) is a serious lung disease. It occurs when the tissue around air sacs (alveoli) in the lungs gets thick and stiff. This makes it harder to breathe. Over time this stiff tissue causes permanent scarring in the lungs, which is called fibrosis.¹

IPF is unpredictable and there is no "normal" with this disease. A person's lung scarring may get worse slowly or quickly. Some people get worse fast, while others remain stable for years. Many people with IPF also have times when their symptoms get much more serious suddenly. These times are called acute exacerbations.^1,2

It is not clear what causes IPF, but people who smoke and those with a family history of IPF may be at higher risk of developing it. There is no cure for IPF, but treatment may slow its progression and help your lungs work better.¹

Medicines for IPF and its complications

There are several drugs your doctor may prescribe if you have IPF. These medicines may help your lungs work better and help prevent exacerbations:^1,2

• Nintedanib (Ofev®) or pirfenidone (Esbriet®) may be given for IPF
• Treprostinil or sildenafil may be given for severe IPF

Unfortunately, some current treatments have significant side effects and no current treatment is a cure.²

Common complications of IPF include acid reflux (gastroesophageal reflux disease or GERD) and microaspirations. Microaspirations occur when tiny bits of stomach acid vomit back up into the lungs, irritating lung tissue. Controlling both these complications may help slow IPF progression.^1-3

Common complications due to scarring (fibrosis) of the lungs from IPF include:^2,4

• Pneumonia
• Collapsed lungs (pneumothorax)
• Pulmonary hypertension
• Blood clots in the lungs (pulmonary embolism)
• Heart failure
• Lung cancer

Other treatments

People with IPF are likely to need help breathing. At first, extra oxygen may only be needed during exercise or other strenuous activities. Later, it may be needed constantly and include:^2,4,5

• Oxygen therapy (oxygen tank)
• Pulmonary rehabilitation
• Ventilator support

Some people with IPF may qualify for a lung transplant. Even though such a transplant is a difficult procedure for someone with IPF, it adds about 5 years of life.²

People with IPF can do several other things to improve their breathing and quality of life, such as:^1,2,4,6

• Get recommended vaccinations, especially for diseases that affect the lungs or breathing.
• Stop smoking, if you smoke.
• Get regular check-ups for close monitoring.
• Alert your doctor to suddenly worse symptoms.
• Exercise as much as possible to maintain strength and lung function.
• Eat smaller meals more often. A full stomach can make it harder to breathe.
• If needed, lose weight to take stress off both your lungs and heart.
• Treat any stress, anxiety, and depression through counseling, medicine, or the support of friends and family.
• Avoid high altitudes.
• Avoid going outside when air quality is poor.
• Avoid dusty indoor places.

Unproven treatments

Doctors have tried many drugs over the years to try and find an effective treatment for IPF. None of these drugs are now recommended for IPF:²

• Anticoagulation
• Azathioprine/prednisone/(N) acetylcysteine combination therapy
• Colchicine
• Cyclophosphamide
• Cyclosporine
• Endothelin receptor antagonists
• Interferon-gamma
• Methotrexate
• (N) acetylcysteine
• Penicillamine

Some of these drugs showed mixed results or made no change in people with IPF. Others had significant side effects. Though some may be of use in the future, at this time there is not enough data to prove safety or effectiveness.

Future directions for IPF research

Doctors have found a number of genetic mutations that are linked to IPF. These genetic changes are thought to account for up to 40 percent of the risk of developing IPF. Mutations have been found in the following genes:^1,4

• MUC5B gene, which produces a mucus protein
• SP-C gene, which creates surfactant (a coating needed in the lungs)
• TERT and TERC genes, which create structures that help chromosomes work correctly

Knowing about the genetic mutations that contribute to some IPF cases may help experts find more successful treatments. Many clinical studies are currently underway to find help for people with IPF.