Idiopathic pulmonary fibrosis is a chronic lung disease that causes the tissue in the lungs to become stiff and scarred, making it difficult to breathe naturally. IPF is known as interstitial lung disease, an umbrella term for a large group of disorders that cause scarring in the lungs. The word “idiopathic” is a means of describing that doctors can’t identify the cause of pulmonary fibrosis. IPF affects nearly 100,000 Americans, making it a rare disease. The damage caused by IPF is progressive and irreversible.

1. IPF causes
The causes of IPF are not fully understood and doctors are unsure why some people get it, hence the idiopathic label. There are a few risk factors that might make some people more likely than others to contract this disease, including cigarette smoking, having acid reflux, age, and breathing in wood or metal dust at work or home. Research has also shown that genetics may play a part. Nearly 20% of IPF patients have another family member with an interstitial lung disease.

2. IPF symptoms
There are patients with IPF that may not notice symptoms and may only notice when the scarring in the lungs gets worse. As the disease progresses, patients may notice symptoms like a dry, hacking cough that doesn’t go away and shortness of breath while walking or performing other activities. Other common symptoms include fatigue, aches in joints and muscles, unintentional weight loss, and clubbing of the fingers and toes.

The symptoms of IPF share many of the same signs as other lung diseases, so it might take time to get the right diagnosis. A pulmonologist is a doctor that treats lung problems and can evaluate and treat the condition.

3. IPF treatments
Treatments for IPF will make it easier to breathe, but it won’t cure the disease. Doctors may use medication, such as nintedanib and pirfenidone have been used to slow down lung damage and scarring. Oxygen therapy includes breathing through a mask or prongs to boost oxygen in the blood. Some patients only use it when they sleep or exercise, but others may need to use it all day long. Working with a team of doctors, nurses, and therapists can help with pulmonary rehabilitation to manage symptoms. Working with a team allows the patient to work on their general health, such as exercising, healthy eating, conserving energy, and stress relief. IPF patients can even be considered for a lung transplant, but doctors usually recommend it for patients whose illness progresses very quickly.