What is Idiopathic Pulmonary Fibrosis (IPF)?

Apart from diseases of the lungs such as asthma, COPD, pneumonia, bronchitis, and cancer, there are diseases in which the connective tissue of the lungs are widely involved, which are neither infectious nor cancerous. These are called interstitial lung diseases. Idiopathic pulmonary fibrosis (IPF) is the most commonly seen of these diseases. profdromerdeniz.com. IPF is characterized by the hardening and loss of elasticity of the lungs, a condition in which there is an increase in connective tissue in the lungs called fibrosis. This increase in connective tissue disrupts the structure of the lung tissue. It usually occurs in older ages. It starts especially from the lower regions and subpleural (under the lung membrane) areas of the lungs. IPF is a disease that usually starts insidiously and progresses slowly. The most important symptoms of IPF are shortness of breath and dry cough. The fingers of patients with IPF may also have clubbing. profdromerdeniz.com

What causes IPF?

IPF is a disease of unknown cause, but a significant proportion of patients have a history of smoking. Many factors such as environmental factors and viruses were suspected in the etiology of the disease but a cause could not be found. profdromerdeniz.com. Concomitant gastroesophageal reflux disease is very common in patients with IPF. 

How is IPF diagnosed?

The most important diagnostic modality for the diagnosis of IPF is thorax HRCT (high resolution computed tomography). Findings suggestive of IPF can be observed in thorax HRCT. In some patients, chest HRCT findings are typical for IPF, in such patients only chest HRCT findings are sufficient for the diagnosis. In such patients, after excluding diseases such as pulmonary involvement of rheumatoid arthritis, asbestosis, which can have a similar appearance, a diagnosis of IPF can be made without the need for biopsy. profdromerdeniz.com. Lung biopsy is required in patients with findings suggestive of IPF but not typical IPF findings. There may also be cases in which a definitive diagnosis cannot be made despite lung biopsy. Pulmonary function tests (PFTs) of patients with IPF may be within normal limits in the early stages of the disease, and as the disease progresses, the lungs become unable to expand thus PFTs indicate a restrictive pattern. This is due to fibrosis in the lungs and loss of elastic structure. profdromerdeniz.com. The fibrous parts of the lungs look like honeycombs, normal lung tissue cannot be mentioned in these parts, so these parts of the lung do not perform respiration.

Is there a cure for IPF?

There is no known, definitive treatment for IPF. Because the diseased areas of the lungs are structurally destroyed, these areas cannot be cured, but there are drug treatments that help slow the progression of the disease in intact lung areas. profdromerdeniz.com. If the disease is very advanced, there is no sense in giving these treatments. Drugs used in the treatment of IPF are pirfenidone  and nintedanib. Lung transplantation is the last option in the treatment of patients with IPF. IPF patients can be candidates for lung transplantation if they meet the requirements for transplantation. profdromerdeniz.com. However, one of the important points to be aware of is that 1-year survival of patients who have undergone lung transplantation is around 85%, and 5-year survival is around 55-60%. Therefore, one of the most important goals in the treatment of patients with IPF can be considered as prolonging the time that patients will go to lung transplantation. profdromerdeniz.com.

Legal Disclaimer: The medical information on this website can only be considered as a source of information, cannot be considered as any medical advice, cannot be used for any diagnosis or treatment purposes, or this information cannot be relied upon for diagnosis or treatment purposes. The medical information on this website is not intended for patient education and cannot create a patient doctor relationship. Please consult your doctor for any health-related decision or medical advice. profdromerdeniz.com