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HYGEIA Hospital Tirana
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Idiopathic Pulmonary Fibrosis


Idiopathic Pulmonary Fibrosis: A unique respiratory condition

Idiopathic pulmonary fibrosis is a condition with an unknown cause and life-threatening consequences for the patient. Diagnosis is quite hard, since the disease manifests common symptoms. So what is it exactly and how is it treated?

Idiopathic pulmonary fibrosis (IPF) is a condition that exclusively affects the lungs and no other body systems (e.g. digestive, musculoskeletal, etc). The cause of the disease is unknown. Without excluding any form of hereditary burden (since there have been some cases of familial conditions), the disease is not considered hereditary. It most frequently affects smokers over the age of 50, but on rare occasions it has been observed in individuals under 45.

Firstly, IPF must be clearly distinguished from the general term “pulmonary fibrosis”, as well as the terms “fibrotic lung diseases” and “cystic fibrosis”. This is because fibrotic lung diseases do not point to IPF, but are usually the result of an old respiratory infection, bronchiectasis or tuberculosis. As opposed to IPF, these conditions do not progress over time.

Furthermore, the general term “pulmonary fibrosis” does not necessarily mean idiopathic pulmonary fibrosis, but may mean development of scar tissue as a result of environmental factors (inhalation of irritating gases), medications or infection of the lung on the grounds of collagen disease.

Finally, cystic fibrosis is not fibrosis per se, but a hereditary disorder marked by cystic bronchiectasis of the lung as a result of frequent respiratory infections.

Symptoms
In IPF, the air sacs (which is where the gases are exchanged: oxygen and carbon dioxide) are replaced by fibrotic (scar) tissue, which limits their ability to exchange gases. This development from normal air sacs to scar tissue does not take place similarly in all patients. In some, the disease develops slowly, takes years and does not progress significantly. In others, it progresses in waves, in that it develops very fast for a while and then it stabilizes, while in others it develops progressively over time.

The symptoms for patients with IPF are a dry cough and progressive dyspnea. Given that the onset of dyspnea is almost always very gradual, patients are not often aware they are experiencing dyspnea, since their mobility is gradually limited. They only actually realize their difficulty in breathing at rest. If, however, they were to think back to their mobility a year ago compared to currently, they would realize they had been suffering from dyspnea.

Patients often attribute their cough and dyspnea to a cold and take antibiotics without prescription, which is forbidden, as this may lead to the development of resistant bacteria. At other times, they take cortisone is large doses. This again is risky, since apart from the underlying feeling of euphoria it causes, cortisone does not affect disease progression, while frequent administration renders the body prone to severe infections and tuberculosis.

Diagnosis & Treatment
IPF is diagnosed by a team of specialists, including a pulmonologist, a radiologist (who studies the high-resolution chest CT) and in some cases an anatomical pathologist who specializes in this disease (if a lung biopsy is required).

Patients are monitored by a pulmonologist who performs a physical exam, spirometry (which measures the volume of air inhaled), diffusion (which determines the ease of gas exchange, i.e. oxygen and carbon dioxide), oximetry at rest or when walking (which monitors whether oxygen saturation is adequate) and a high-resolution chest CT.

Treatment is based on the instructions of the pulmonologist. If patients also suffer from chronic bronchitis (due to smoking), asthma or infection, all of which can aggravate their dyspnea, the doctor may prescribe the appropriate medications. Oxygen is administered only if necessary and its action may be beneficial both for the lungs and the heart. If patients are also suffering from gastroesophageal reflux disease, this must be treated as it appears to aggravate the progress of IPF.

According to the latest international guidelines, cortisone is not recommended, unless the condition is exacerbated (worsening of dyspnea in a short while).

Pirfenidone and nintedanib are new medications that demonstrate anti-fibrotic properties, but administration must be monitored by a physician. They seem to improve survival and reduce exacerbations of the disease.

In select patient groups, lung transplantation may be performed toward the end stages of the disease.