Idiopathic Pulmonary Fibrosis – What You Need To Know?

IPF irreversibly damages the patient’s lungs and causes the patient to experience shortness of breath – initially during exercise, and as the disease progresses, also during everyday, simple activities such as brushing teeth or talking. Lech Karpowicz has been suffering from IPF for several years. Initially, the doctor diagnosed him with pneumonia and treated him with antibiotics. After three years, he finally got an X-ray and then a tomography. Only then was the correct diagnosis made. The first symptoms of the condition are usually a persistent dry cough, shortness of breath with unresponsive physical exertion, and bilateral crackling sounds heard on auscultation of the chest, which may suggest pneumonia.                                            

Although the cause of IPF remains unknown, growing evidence suggests that genetic factors may play a significant role in the development of the disease. It is also supposed that the factors contributing to its occurrence may include: smoking, previous viral infections, gastroesophageal reflux, contact with chemicals, bird droppings and other harmful environmental factors (e.g. due to the profession). According to experts, currently around 900 people suffer from IPF in Poland. Every year, on average, about 250 new patients are added. However, due to low awareness of the disease, this number most likely does not reflect the scale of the problem. For comparison, in Great Britain it is diagnosed in about 5 people each year. IPF mainly occurs in patients over the age of 000. Considering that Poles are among the fastest aging societies in the EU, the problem of IPF will continue to grow.

In Poland, the low awareness of the disease among doctors and the diagnosis that requires the cooperation of many specialists make it difficult to make a proper diagnosis quickly. In addition, it requires the exclusion of other causes of pulmonary fibrosis and the performance of a number of tests, such as medical interview combined with a detailed examination, spirometry, high-resolution computed tomography, and sometimes also lung biopsy or bronchoscopy. Pulmonologist Dr. Katarzyna Lewandowska tells about her patient who was previously treated for bronchial asthma. Only when she began to choke in a state of rest did she go to the pulmonologist. Meanwhile, the doctor emphasizes, the earlier the patient is under professional care, the better his prognosis.

At present, it is impossible to predict how quickly the disease will progress in a patient. IPF cannot be cured, but it does manage to alleviate its symptoms, slow its progression, and extend the patient’s life. Only every fifth patient survives 5 years after being diagnosed with the disease. The estimated survival rate in IPF is 20–40%. after 5 years, which means that the mortality in IPF is higher than for many malignancies, including breast, ovarian or colon cancer. – There are patients whose time from diagnosis to death is very short. In others, it takes a long time to develop – says prof. Jan Kuś, head of the XNUMXst Department of Lung Diseases, Institute of Tuberculosis and Lung Diseases in Warsaw. Patients die primarily from respiratory failure, one in five develops lung cancer, and some from comorbidities.

Until recently, after diagnosis, patients were given medications to alleviate the symptoms of the disease and treat its complications, including oxygen therapy, antibiotic therapy, antitussive drugs and expectorants. At present, the optimal methods of treatment include medications that inhibit pulmonary fibrosis, oxygen for breathing, and pulmonary rehabilitation. Pharmacological therapy to inhibit pulmonary fibrosis is available in the European Union countries, in Poland it is not yet reimbursed.

A therapeutic alternative is lung transplantation. Transplantation takes place in less than 5 percent. patients with IPF. It is estimated that the survival rates after this procedure in patients with IPF are: 95%. after 30 days, 73 percent. after 1 year, 56 percent. after 3 years and 44 percent after 5 years. About 160 lung transplants are performed annually in Poland. One third of them concern patients with IPF. 60 percent of these patients do not survive the operation for 5 years. Those who qualify for a lung transplant but do not receive it die within a year. Abroad, the transplant costs about 200 euros. However, there are also a shortage of donors.

For as yet unknown reasons in the course of this disease, cells in the tissue surrounding the alveoli become damaged and begin to die. The body tries to repair the damage and the process results in fibrosis, i.e. scarring and hardening of the lung tissue. This disrupts the functioning of the alveoli in the lungs, which cannot properly expand, leading to a reduction in the amount of oxygen reaching the blood and tissues. This leads to organ dysfunction and disability. Each year, approximately 35 new cases of IPF are diagnosed in Europe. All forecasts emphasize that this number will increase year by year.