What is idiopathic pulmonary fibrosis and the key sound that can help detect it

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On auscultation, healthcare professionals can identify a characteristic sound caused by the disease.

It is known that Covid-19, the disease caused by the new coronavirus, is not “a flu” and that it can cause significant damage to health in severe cases. One of the serious consequences that occurs in some people is pulmonary fibrosis, a process of scarring of the lung, which hardens the tissue and reduces the respiratory capacity. In people in whom the condition occurs as a result of SARS-CoV-2 infection, the cause is well known.

But there are people who live with this chronic and progressive pathology in whom the origin cannot be found, which is why it acquires the name of idiopathic pulmonary fibrosis (IPF), a rare disease that in Argentina would affect between 5,000 and 6,000 people, according to projections based on records from the United States and Europe, since there are no local epidemiological data. However, specialists warn that there is a great underdiagnosis. With the aim of generating greater awareness about this disease, today is the World Day of Idiopathic Pulmonary Fibrosis.

The symptoms of IPF, which in addition to dyspnea (shortness of breath) include a persistent dry cough, chest discomfort and fatigue are common to those of much more frequent pathologies such as COPD, asthma or those originating in the heart. so those who live with it usually go through several doctors and delay almost two years to find the correct diagnosis, from which you can start a treatment that slows down the progression of the disease. “About half receive a wrong initial diagnosis,” says Gabriela Tabaj, coordinator of the Interstitial Diseases Clinic at the Antonio Cetrángolo Thorax Hospital.

IPF affects men more than women, and is seen more frequently in smokers or former smokers. “Although it is a disease characterized by abnormal scarring of the lung tissue against a injury not yet determined (idiopathic refers to the fact that its origin is unknown), risk factors such as smoking, exposure to toxic particles and certain genetic mutations have been identified as there are familiar forms of the disease, “explains Tabaj.

When to suspect it? It manifests as shortness of breath or progressive dyspnea that start in exercise and in more advanced cases it can appear at rest. In addition, the patient may report a dry cough. The incidence increases with age, typically presenting between the ages of 60 and 70, rarely occurring before 50, the doctor says.

“In the physical examination, the presence of sounds called crepitant rales of the velcro type draws attention to the auscultation, since they evoke the sound of the open with a bristle. These sounds are early signs of the disease, “he details.

To make a correct diagnosis it is key to have a chest computed tomography resolution, but in a minority of cases the tomography is not conclusive and a lung biopsy.

An exposure origin (inhalation of toxic particles) and autoimmune diseases must always be ruled out. In addition, it is essential to have tests such as spirometry and carbon monoxide diffusion capacity to objectify the degree of lung function compromise, says the doctor, who is the Argentine coordinator of REFIPI, the first continental registry of the disease worldwide, in which 14 Latin American countries participate.

Specialists in this rare disease that greatly limits the quality of life of those who live with it emphasize that early diagnosis is key, since most progress progressively with decline in lung function. The sooner the diagnosis is made, the sooner a specific treatment can be started.

“Although we currently have specific pharmacological treatments that have shown modify the course of the disease, the global approach of the person is key; and at this point the respiratory rehabilitation it has proven to be extremely beneficial. It is also important not to neglect the monitoring of associated conditions such as reflux, sleep apnea, diabetes, heart failure, depression and evaluation for oxygen therapy. Monitoring by interdisciplinary teams it is essential in this type of disease “, concluded Tabaj.

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