In idiopathic pulmonary fibrosis the lung tie is thickened. Scarring of the tie in the lungs.
IPF is a rare serious disease in which the ties of the lungs are affected. Normally the tie is soft and flexible. In IPF the lung tie is damaged, it becomes scarred. This change is called fibrosis. Once lung tie is damaged in this way, it does not heal again.
What is IPF?
Facts about IPF
1 What are the causes of IPF?
It is not known exactly what causes IPF. Hence the word "idiopathic", which means "unknown cause".
2 Does IPF occur frequently?
No, it is a rare disease and many people have never heard of it. About 14.000 people in Germany have IPF. The average age of newly diagnosed patients is 66 years old. People under the age of 50 are rarely diagnosed with IPF. 1
3 Can IPF be cured?
In some patients, a lung transplant can be performed. This is the only way to cure IPF. In addition, there are medications specifically approved for IPF that can slow the progression of the disease. Many affected people are thus able to do the things that are important to them as long as possible.
Diagnosis of IPF
In idiopathic pulmonary fibrosis, the scarring of the lungs usually begins slowly, so that it is not even noticed at first. It does not cause symptoms until the later stages.
Signs of the disease include:
Cough (dry, irritating cough without sputum)
Breathlessness (shortness of breath initially during physical activity)
Weight loss (gradual, unintentional weight loss)
Fatigue (impaired performance)
Drumstick fingers (widening and rounding of the fingertips and toes)
Pain (especially in muscles and joints
Especially at the beginning of the disease, the symptoms are still non-specific, so various tests must be performed to make the diagnosis of IPF. The earlier the diagnosis is made, the sooner fibrosis of the lung tie can be treated, slowing the progression of the disease. A clear diagnosis is a prerequisite for optimal therapy.
The basis of the diagnosis is the previous medical history and a thorough examination. Listening to the lungs (auscultation) is of great importance here. The doctor can often hear a rattle in both lungs, typical of IPF, at the end of the inhalation:
This sound is reminiscent of the opening of a Velcro fastener. Another indication of lung disease are thickened fingertips ("drumstick fingers") and enlarged "watch glass nails. Other tests are also available, including X-rays or high-resolution computed tomography ("HRCT") of the lungs, a bronchoscopy, lung function tests or a lung biopsy, in which tie is removed from the lungs. Once the diagnosis of IPF is confirmed, patients must continue to attend regular check-up appointments where the progression of the disease is monitored. Examination procedures include listening to your lungs, lung function tests, and blood tests to check the function of your liver and kidneys.
How IPF is diagnosed?
Therapy of IPF
The scarring of your lungs caused by IPF cannot be reversed. For this reason, it is extremely important that your healthy tie is preserved for as long as possible. Decide on the right treatment for you together with your doctor during the consultation. Discuss your treatment options with your doctor and then take the path that's right for you. There are two relatively new drugs approved specifically for treating IPF. Their mode of action slows down the progression of the disease. Affected people have the possibility to continue doing the things that are important to them. Side effects may occur during treatment. Your doctor can usually help you here.
A lung transplant is currently the only cure for IPF. However, it is only an option for very few patients. It is also associated with risks. Most importantly, a matching donor lung must be found for you, which can take a very long time. Furthermore, you must be in good physical condition. Your doctor can give you more information about organ transplantation and the procedures involved. Your doctor may offer you other treatment options to help manage the symptoms of IPF in your daily life. These include, for example, pulmonary rehabilitation and oxygen therapy. These offers can be helpful in a supportive way, but they do not slow down the progression of the disease.