Idiopathic pulmonary fibrosis
Overview
Idiopathic pulmonary fibrosis (IPF) is a condition in which the lungs become scarred and breathing becomes increasingly difficult.
Symptoms
The symptoms of IPF tend to develop gradually and get slowly worse over time. Symptoms can include: shortness of breatha persistent drycoughtirednessloss of appetite andweight lossrounded and swollen fingertips (clubbed fingers) Many people ignore their breathlessness at first and blame it on getting old or being out of shape. But eventually even light activity such as getting dressed can cause shortness of breath.
Causes
In people with IPF, the tiny air sacs in the lungs (alveoli) become damaged and increasingly scarred. This causes the lungs to become stiff and makes it difficult for oxygen to get into the blood. The reason this happens is not clear. Idiopathic means the cause is unknown. IPF has been linked to: exposure to certain types of dust, such as metal or wood dustviral infectionsa family history of IPF – around 1 in 20 people with IPF has another family member with the conditionacid refluxsmoking But it's not known whether some of these factors directly cause IPF.
Treatment
There's currently no cure for IPF, but there are several treatments that can help relieve the symptoms and slow down its progression. Treatments include: self care measures, such as stopping smoking, eating healthily and exercising regularlymedicines to reduce the rate at which scarring worsens, such as pirfenidone and nintedanibbreathing oxygen through a mask – you can do this while you're at home or while you're out and aboutexercises and advice to help you breathe more easily (pulmonary rehabilitation)a lung transplant – this is suitable in a few cases, although donor lungs are rare Find out more about how idiopathic pulmonary fibrosis is treated
Prevention
Stop smoking; wear mask in dusty environments.
When to see a doctor
See GP if: breathless for no reason; persistent cough (>3 weeks); clubbed fingers.