Pulmonary Fibrosis Medications | American Lung Association

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Pulmonary Fibrosis Medications

Depending on the type of pulmonary fibrosis you have, there may be medicine available to treat your disease. Only your medical provider can determine if these medications may be beneficial for you.

FDA-approved Drugs for IPF

Currently, two drugs are FDA-approved for treatment of idiopathic pulmonary fibrosis (IPF), which is the most common form of PF. These include nintedanib (Ofev®) and pirfenidone (Esbriet®). These medications are called anti-fibrotic agents, meaning that they have shown in clinical trials to slow down the rate of fibrosis or scarring in the lungs. These drugs are approved for patients with mild, moderate and severe IPF. While neither medication is recommended over the other, one may work better for you. Your doctor will work with you to prescribe treatment tailored to your needs. While taking these medications, you will be monitored closely for any serious side effects. Monitoring will include routine blood work to make sure your liver remains healthy. Ask your doctor for a referral to a palliative care specialist who can help you manage any side effects from the drugs.

Drugs to Reduce Inflammation

There are certain types of PF (excluding IPF) that respond to treatment with steroids. Steroids, also known as glucocorticoids, are a class of medication that reduces inflammation (swelling). Corticosteroids can have harmful side effects, especially when used long term. You will need to be carefully monitored if you are on long-term steroids. Other groups of medications prescribed to reduce inflammation include azathioprine, cyclophosphamide and mycophenolate mofetil (Cellcept®, Myfortic®), which work by suppressing the immune system.

Drugs to Treat Acid Reflux

Doctors may recommend anti-acid medications to treat gastroesophageal reflux disease (GERD), a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis. There are different types of anti-acid medications (e.g., Proton pump inhibitors including Prilosec OTC® and  Nexium® as well as H2-Blockers including Zantac® and Pepcid®) that are prescribed to help block the formation of or minimize the amount of acid in the stomach. By doing so, it helps to reduce the amount of acid from the stomach that enters the lungs.

Drugs to Treat Cough

A dry, hacking cough is a common symptom of PF. Treating cough is mostly trial and error. What works for one patient may not work for another. Some treatment options for cough include:

  • Over the counter cough medicine like Robitusson® and cough drops
  • Prescription cough medicine like hydrocodone (Tussionex PennKenetic®) and benzonatate (Tessalon Perles®)
  • Thalidomide (Thalomid® ) may be used for serious cases of cough in some patients.
  • There are ongoing clinical trials for PF drugs, including ones to help relieve cough. Visit clinicaltrials.gov to see if any are available near you or ask your doctor.
    Webpage Resource

    Clinical Trials and Pulmonary Fibrosis

    Learn more
    Webpage Resource

    How Do I Manage Symptoms and Side Effects of Pulmonary Fibrosis?

    Learn more

    Reviewed and approved by the American Lung Association Scientific and Medical Editorial Review Panel. Last reviewed February 5, 2018.

    Page Last Updated: March 13, 2018

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