Boehringer Ingelheim Statement on ATS/ERS/JRS/ALAT Guidelines for Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis

Boehringer Ingelheim welcomes the new guidance issued by American Thoracic Society, European Respiratory Society, Japanese Respiratory Society and Asociación Latinoamericana de Tórax, which will help inform disease diagnosis and management strategies for people living with progressive pulmonary fibrosis (PPF) with interstitial lung disease (ILD) and share updated information on idiopathic pulmonary fibrosis (IPF).

The guidelines provide clarity around how to define PPF, including clinical, physiological and radiological criteria that may result in diagnosis, determination of progression and initiation of treatment. Identification of progression and initiation of treatment are critical, as lung function decline can be unpredictable in patients with PPF. Loss of lung function is cumulative and even small losses can make a big impact over time. Approximately one out of four patients may develop PPF.

These new guidelines also suggest OFEV (nintedanib) capsules for the treatment of PPF in patients who have failed standard management for fibrotic ILD, other than IPF. This is a conditional recommendation. OFEV has been FDA approved for over 7 years, has a proven mechanism of action, a comprehensive efficacy and safety profile, and remains the only treatment approved to treat adults with chronic fibrosing ILDs with a progressive phenotype.

In addition, the new guidelines now suggest both nonpharmacological and pharmacological therapies, including OFEV, for people living with IPF, one of the most common forms of ILD.

What is OFEV?

• OFEV is a prescription medicine used:
  • to treat adults with a lung disease called idiopathic pulmonary fibrosis (IPF).
  • to treat adults with a long lasting (chronic) interstitial lung disease in which lung fibrosis continues to worsen (progress).
  • to slow the rate of decline in lung function in adults with systemic sclerosis-associated interstitial lung disease (SSc-ILD) (also known as scleroderma-associated ILD).
• It is not known if OFEV is safe and effective in children.

Important Safety Information

What is the most important information I should know about OFEV?

OFEV can cause harm, birth defects, or death to an unborn baby. Women should not become pregnant while taking OFEV. Women who are able to become pregnant should have a pregnancy test before starting treatment and should use highly effective birth control at the start of treatment, during treatment, and for at least 3 months after your last dose. Talk with your doctor about what birth control method is right for you during this time. Birth control pills may not work as well in women having vomiting, diarrhea, or other problems reducing the drug absorption. If you have any of these problems, talk with your doctor about what highly effective birth control method is right for you. If you become pregnant or think you are pregnant while taking OFEV, tell your doctor right away.

What should I tell my doctor before using OFEV?

Before you take OFEV, tell your doctor about all of your medical conditions, including if you have:

• liver problems.
• heart problems.
• a history of blood clots.
• a bleeding problem or a family history of a bleeding problem.
• had recent surgery in your stomach (abdominal) area.

Tell your doctor if you:

• are pregnant or plan to become pregnant.
• are breastfeeding or plan to breastfeed. It is not known if OFEV passes into your breast milk. You should not breastfeed while taking OFEV.
• are a smoker. You should stop smoking prior to taking OFEV and avoid smoking during treatment.

Tell your doctor about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements such as St. John’s wort.

What are the possible side effects of OFEV?

OFEV may cause serious side effects.

TELL YOUR DOCTOR RIGHT AWAY if you are experiencing any side effects, including:

• Liver problems. Unexplained symptoms may include yellowing of your skin or the white part of your eyes (jaundice), dark or brown (tea-colored) urine, pain on the upper right side of your stomach area (abdomen), bleeding or bruising more easily than normal, feeling tired, or loss of appetite. Your doctor will do blood tests to check how well your liver is working before starting and during your treatment with OFEV.
• Diarrhea, nausea, and vomiting. Your doctor may recommend that you drink fluids or take medicine to treat these side effects. Tell your doctor if you have these symptoms, if they do not go away, or get worse, and if you are taking over-the-counter laxatives, stool softeners, and other medicines or dietary supplements.
• Heart attack. Symptoms of a heart problem may include chest pain or pressure, pain in your arms, back, neck, or jaw, or shortness of breath.
• Stroke. Symptoms of a stroke may include numbness or weakness on one side of your body, trouble talking, headache, or dizziness.
• Bleeding problems. OFEV may increase your chances of having bleeding problems. Tell your doctor if you have unusual bleeding, bruising, wounds that do not heal, and/or if you are taking a blood thinner, including prescription blood thinners and over-the-counter aspirin.
• Tear in your stomach or intestinal wall (perforation). OFEV may increase your chances of having a tear in your stomach or intestinal wall. Tell your doctor if you have pain or swelling in your stomach area.
• Increased protein in your urine (proteinuria). OFEV may increase your chances of having protein in your urine. Tell your doctor if you have any signs and symptoms of protein in the urine such as foamy urine, swelling, including in your hands, arms, legs, or feet, or sudden weight gain.

The most common side effects of OFEV are diarrhea, nausea, stomach pain, vomiting, liver problems, decreased appetite, headache, weight loss, and high blood pressure.

These are not all the possible side effects of OFEV. For more information, ask your doctor or pharmacist. You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

Please see full Prescribing Information, including Patient Information.

CL-OF-100056 01.18.2022

For more than a century, Boehringer Ingelheim has remained committed to addressing the significant unmet needs of people affected by serious respiratory diseases, including PPF with ILD. We remain steadfast in our commitment to redefine patient care and are encouraged to see global guidelines continue to evolve to help improve treatment strategies for those living with these devastating diseases.

About Boehringer Ingelheim
Boehringer Ingelheim is working on breakthrough therapies that improve the lives of humans and animals. As a leading research-driven biopharmaceutical company, the company creates value through innovation in areas of high unmet medical need. Founded in 1885 and family-owned ever since, Boehringer Ingelheim takes a long-term perspective. Around 52,000 employees serve more than 130 markets in the three business areas, Human Pharma, Animal Health, and Biopharmaceutical Contract Manufacturing.

Learn more at www.boehringer-ingelheim.com/us