Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis (hardening of the lungs) is a progressive lung disease that causes permanent damage to the lungs.

Idiopathic Pulmonary Fibrosis (IPF) is a lung disease of unknown cause, mostly seen in older adults, characterized by thickening of the walls of the air sacs (alveoli) in the lungs with scar-like tissue formation (scarring), presenting with symptoms of long-term cough, shortness of breath and fatigue. 

Normal healthy lung tissue is soft and flexible, allowing easy breathing. In IPF, the lung tissue is damaged, becoming scarred (also known as scar tissue) and hardened over time. This process is called fibrosis.

The scar tissue (scarring or tissue scarring) that forms in the lungs of an IPF patient hardens the lungs, making it difficult for the lungs to fill with air and reduces the lungs' ability to transport oxygen into the body. The cause of the lung scarring is not exactly known.

The word "idiopathic" in the disease's name means that the cause of the disease is not clearly known. However, some risk factors that may be associated with Idiopathic Pulmonary Fibrosis have been identified.

These risk factors may include genetic mutations, advanced age, male gender, smoking, environmental and occupational exposures, chronic microaspiration, and viral and bacterial infections.

The two most important of these are being current or ex-smoker and occupational or environmental exposure to certain types of harmful dust. Exposure to metal, wood, stone or farm dust may damage the lungs and increase the risk of developing Idiopathic Pulmonary Fibrosis. Some other risk factors include:

• Pulmonary diseases caused by certain viruses

• Gastroesophageal reflux disease: The gastric acid from the stomach leaking up into the esophagus, and accidental inhalation of the biliary, pancreatic or small-intestinal contents may damage the air sacs in the lungs.

• Environmental pollutants and dusts

• Genetic predisposition, family history of Idiopathic Pulmonary Fibrosis: This risk factor is seen in only 5% of all IPF patients.

Symptoms resulting from lung damage caused by IPF start rapidly. In some people, the disease progression is slower. It is difficult to exactly predict the progression of the disease. Therefore, doctors cannot be sure how fast IPF will progress in a patient.

Many factors can affect the prognosis of the disease, such as other health problems or advanced age. The main symptoms of the disease are as follows:

• Shortness of breath

• Non productive persistent cough

• Finger clubbing

• Loss of appetite and weight

• Fatigue, asthenia

• Pain in muscle and bones

Since complaints such as cough and shortness of breath seen in IPF are similar to complaints seen in other lung diseases such as COPD (Chronic Obstructive Pulmonary Disease), the diagnosis of IPF is difficult and may take time.

During an examination, hearing crackle-like respiratory sounds (rales) with deep breathing in lung sounds and non-painful swellings at the tips of the fingers called 'finger clubbing' are important findings of IPF. The diagnosis is based on pulmonary function tests, blood oxygen level measurement, chest radiography, echocardiography (heart radiography), bronchoscopy, 6-minute walk test and high-resolution lung tomography (HRCT) performed after physical examination. In some cases, a lung biopsy may be required.

Today, there is no drug option that completely cures the disease, but antifibrotic treatments are available as treatment options that will allow the effects of the disease to be managed. The principal goal of treatment is to alleviate the patients' complaints as much as possible and to slow down the progression of the disease. In addition to these treatments, supportive methods such as pulmonary rehabilitation, regular exercise, breathing techniques and oxygen therapy help to reduce complaints and help patients live with IPF.

An important treatment option that prolongs survival and improves quality of life in patients with IPF is lung transplantation. Currently, lung transplantation, which is the only treatment option that can provide cure in IPF, is only suitable for a very small number of patients.

In order to raise awareness about Idiopathic Pulmonary Fibrosis (IPF) and to provide access to reliable information about the disease, the website,was launched in 2020.

References

1. https://www.nhsinform.scot/illnesses-and-conditions/lungs-and-airways/idiopathic-pulmonary-fibrosis#causes-of-idiopathic-pulmonary-fibrosis. Last access date 09.06.2023

2. Meltzer EB, et al. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008;3:8.

3. American Thoracic Society Patient Information Series. Am J Respir Crit Care Med Vol. 183, P1-P2, 2011

4. Türk Toraks Derneği̇ İdi̇yopati̇k Pulmoner Fi̇brozi̇s (İPF)  Tanı Ve Tedavi̇ Uzlaşı Raporu 2018

5. Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788–824.