Most people don’t notice the changes to their lungs at first and may think they’re unfit or tired. This can be true but it’s important to be aware of your body’s capabilities and notice any changes, especially if you have any risk factors for developing IPF.

There are a variety of symptoms you may experience with IPF and some of these will worsen as your disease progresses. Keep in mind that there are lifestyle changes and different treatments that can help you manage them. Symptoms can also vary from one person to another, so your symptoms may not be the same as somebody else who has IPF.^3,6-9

Common signs and symptoms of idiopathic pulmonary fibrosis:³

Cough is the most common symptom of IPF and may start long before the disease is diagnosed. It most commonly is a dry cough but occasionally affected people can bring up small amounts of sputum (phlegm). Shortness of breath, especially with physical activity develops as the disease gets worse.

Such symptoms can also be present in other lung diseases (and heart problems), and so can be difficult to tell apart.

Idiopathic pulmonary fibrosis shares symptoms with many other diseases, meaning diagnosis can take a long time

Diagnosing IPF can take a long time (up to two years or more), as many people will have had a lot of different tests before being diagnosed. Although this can be frustrating for patients,³ remember that doctors are always looking for ways to shorten the time to diagnosis.

When looking for the correct diagnosis of IPF, your treatment team will need to rule out all other diseases. They will check for different symptoms, ask about your medical history and perform some tests to reach a final diagnosis. This is why you may have seen many different types of doctors and specialists before being diagnosed with IPF.^3,11

When diagnosing IPF, your treatment team will look at many areas of your health. For example, they will ask you about your work history, family medical history, medications that you are taking, and whether you smoke or have ever smoked. 

Recognising IPF early is key to getting the help you need. Ask your doctor for a lung examination, it can give your doctor a better understanding of your lung health. "Velcro-like" crackles will typically be heard when the doctor listens to the lungs with a stethoscope. However, other tests, particularly breathing tests and CT scans, will be needed to make a clear diagnosis.^3,11 Your doctor, or you, might also notice finger clubbing (widening and rounding of the tips of fingers or toes with a spongy sensation).

Antifibrotic medicines are one of the most recent treatment options available for IPF that have been shown to improve patients’ outcomes.¹³

Antifibrotics are medicines which aim to slow disease progression by slowing down the process that causes scarring in the lungs. They can be effective, so they will likely form a key part of your treatment plan. Speak to your doctor to find out if they are a suitable treatment option for you.¹³ In many countries, there are two antifibrotic medicines available to treat IPF—nintedanib and pirfenidone.¹³

Your doctor may sometimes prescribe treatments other than antifibrotics. These may aim to improve your symptoms and your general wellbeing and might include pulmonary rehabilitation or oxygen therapy. The treatment team will always consider your individual situation and work with you to decide on the treatment plan best suited to you.^3,13

IPF is a progressive disease, which means that it gets worse over time. However, there is no way of predicting how fast this will happen. The rate at which IPF will progress can also change over time. Some patients may notice few changes in their symptoms, while other patients experience a faster worsening of their symptoms. It is not known why some people’s disease progresses faster than others.³

Some research suggests that half of people with IPF live for about 3 to 5 years after diagnosis. However, these estimates were based on information provided before new treatments for IPF were available.^1,3

With new antifibrotic treatments for IPF, people with IPF can live longer. Although the new treatments cannot reverse the scarring on the lungs, they may slow the progression of the disease.^5,14,15

Your doctor, supported by a wider treatment team of experienced specialists will use your medical history, a physical examination and a combination of different tests you'll be familiar with from diagnosis, to find out how your disease is progressing. Your doctor will then tailor the best treatment plan for you.^3,13Ask your healthcare professional about your treatment options, including pulmonary rehabilitation, which could help you manage your condition.¹⁰

It’s important that your healthcare professional checks your lung health regularly so that any changes can be managed quickly.¹⁶

Unlike many other chronic diseases, there are no clear-cut "stages" of IPF. Everyone experiences it differently so it’s best to speak to your doctor who can advise you based on your individual situation.¹³

In the early stages of IPF, you may only notice small changes in your breathing. As the disease progresses, there is a bigger risk of complications or an acute exacerbation occurring. An acute exacerbation is a sudden and rapid worsening of symptoms.^1,17

If you have a high temperature you may think you have a chest infection or are experiencing a flare-up of your condition but it's important you seek medical advice anyway. Acute exacerbations can be life-threatening and for this reason, it is vital that you work with your treatment team to do everything you can to look after your lungs.¹⁷

Many people with IPF can find it hard to manage their thoughts or mental wellbeing. Thoughts can involve feeling down or worried and struggling to cope with the diagnosis. Looking after your mental health is just as important as looking after your physical health. Speak to your treatment team if you feel like you need help managing your mental wellbeing.

You may have other ongoing health conditions that should be managed alongside your IPF

Many people with IPF also have other health conditions which are often called comorbidities, or co-existing conditions. IPF may, or may not, influence these but managing them can be just as important as managing IPF itself.

To manage co-existing conditions people receive medications and care in addition to their treatment for IPF. Some of the most common co-existing conditions people with IPF may have include:^18,19

• Chronic obstructive pulmonary disease (COPD): A lung disease that causes the flow of air into and out of the lungs to be restricted. This makes it harder to breathe

• Gastroesophageal reflux disease (GERD): A stomach condition that causes stomach acid move up into the food pipe (oesophagus), leading to heartburn

• Obstructive sleep apnea: A condition where breathing stops and starts during sleep

• Pulmonary hypertension: High blood pressure in the blood vessels that supply blood to the lungs

• Heart conditions such as coronary heart disease, where there is a build-up of plaque in the blood vessels in the heart

• Other conditions: Diabetes, underactive thyroid and hiatal hernia

It is important to remember that every person with IPF is different and may experience some, or none, of these co-existing conditions. Always be aware of any changes in your condition or symptoms and discuss them with your treatment team. They will be able to guide you on your condition and treatments.

Am I alone in how I feel about my diagnosis?

Although IPF is rare, you are not alone. There are other people with IPF like you, all over the world, who are working through the emotional and mental wellbeing challenges of living with IPF. In fact, anywhere from 10-60 people in every 100,000 people are diagnosed with IPF every year in Europe and North America.^1,20