Idiopathic pulmonary fibrosis is a serious, terminally fatal disease characterized by a steady decline in lung function. The term pulmonary fibrosis refers to a scarring of the lung tissue, which leads to an ever-increasing dyspnea and dry cough.
IPF usually occurs in adulthood between the ages of 50 and 70, men are more frequently affected than women. IPF belongs to a group of about 200 lung diseases known as interstitial lung diseases, which are characterized by pulmonary interstitial involvement.
Also alveoli, peripheral airways and vessels are affected. Auscultatory can often be an early-inspirational crackling rattle, especially in the basal lung areas, impress. With advanced disease, a cor pulmonale may appear with the classic signs of right heart failure. The cause of the IPF is unknown.
The participation of environmental factors (cigarettes, exposure to quartz dust, animal husbandry) is possible. Evidence of the pattern of "common pulmonary fibrosis" (Uual) is an integral part of the diagnosis of IPF.
In HRCT (high-resolution computed tomography), this is seen in the lower lobes as peripheral, subpleural, reticulate clouding, along with the typical "honeycomb pattern". Histopathologically, it shows itself by alternating little changed or healthy lung areas with areas of dense fibrotic areas with pitted and honeycomb-shaped changes.
The only effective therapeutic measure is lung transplantation. The median survival time from the time of diagnosis is approximately 2-5 years.