Pulmonary Fibrosis Overview
Pulmonary fibrosis is a disease marked by scarring in the lungs. Tissue deep in the lungs becomes thick, stiff and scarred. The scarring is called fibrosis. As the lung tissue becomes scarred, it interferes with a person’s ability to breathe.
In some cases, the cause of pulmonary fibrosis can be found. But most cases of pulmonary fibrosis have no known cause. These cases are called idiopathic pulmonary fibrosis.
Pulmonary fibrosis can develop slowly or quickly. There is no cure. Many people with the disease live only about three to five years after diagnosis.
Current treatments for pulmonary fibrosis cannot remove scarring that has already occurred. There are a number of new treatments being studied for pulmonary fibrosis.
There are many resources available to people living with pulmonary fibrosis and their loved ones.
Symptoms of Pulmonary Fibrosis?
Symptoms of pulmonary fibrosis include:
- shortness of breath,
- coughing, and
- diminished exercise tolerance.
Symptoms vary depending on the cause of the pulmonary fibrosis. The severity of symptoms and the progression (worsening) of symptoms over time can vary.
The most common form, idiopathic pulmonary fibrosis, has slow and relentless progression. Early on, patients often complain of a dry, unexplained cough. Often, slow and insidious onset of shortness of breath can set in. With time, dyspnea (shortness of breath) worsens. Dyspnea initially occurs only with activity and is often attributed to aging. Over time, the dyspnea occurs with little or no activity. Eventually, the shortness of breath becomes disabling, limiting all activity and even occurring while sitting still. In rarer cases, the fibrosis can be rapidly progressive, with dyspnea and disability occurring in weeks to months from the onset of the disease. This form of pulmonary fibrosis has been referred to as Hamman-Rich syndrome.
Who is affected by Pulmonary Fibrosis
IPF is a relatively rare condition, although the number of cases has risen in recent years. It is estimated that more than 5,000 cases are diagnosed every year in the UK.
The condition usually first develops in adults aged 50 or above and is thought to be more common in men.
Causes of Pulmonary Fibrosis
Some people get pulmonary fibrosis when they’re exposed to something in their environment, like pollution, certain medicines, or an infection. But most of the time, doctors don’t know what causes IPF. That’s what “idiopathic” means.
You might be more likely to get IPF if you:
- Breathe in wood or metal dust at work or home
- Have acid reflux disease
- Smoke cigarettes
Sometimes, IPF runs in families. Doctors think broken genes could cause the disease in some people. No one knows yet which specific genes may be involved.
Risk factors of Pulmonary Fibrosis
Factors that make you more susceptible to pulmonary fibrosis include:
- Age. Although pulmonary fibrosis has been diagnosed in children and infants, the disorder is much more likely to affect middle-aged and older adults.
- Smoking. Far more smokers and former smokers develop pulmonary fibrosis than do people who have never smoked. Pulmonary fibrosis can occur in patients with emphysema.
- Your occupation. You have an increased risk of developing pulmonary fibrosis if you work in mining, farming or construction or if you’re exposed to pollutants known to damage your lungs.
- Cancer treatments. Having radiation treatments to your chest or using certain chemotherapy drugs makes you more susceptible to pulmonary fibrosis.
- Genetic factors. Some types of pulmonary fibrosis appear to run in families, so a genetic component is suspected.
Treatment of Pulmonary Fibrosis
Treatments for IPF won’t cure the disease, but they can make it easier for you to breathe. Some may keep your lungs from getting worse quickly. Your doctor may recommend a few options:
- Medicine. Two drugs, pirfenidone (Esbriet) and nintedanib (Ofev), are approved to treat IPF. Scientists are still finding out exactly how they work, but they do know these treatments slow down scarring and damage in your lungs.
- Oxygen therapy. You breathe oxygen through a mask or prongs that go in your nose. It boosts the oxygen in your blood so you have less shortness of breath and can be more active. Whether you need to wear oxygen depends on how serious your condition is. Some people with IPF need it only when they sleep or exercise. Others need it 24 hours a day.
- Pulmonary rehabilitation. You work with a team of doctors, nurses, and therapists on ways to manage your symptoms. You might focus on exercise, healthy eating, relaxation, stress relief, and ways to save your energy. You may visit the hospital for a rehab program or do one at home.
