Idiopathic pulmonary fibrosis (IPF) occurs when lung tissue gradually becomes damaged and scarred, becoming thicker and stiffer. This is a pathology, the cause of which has not yet been established, but it is assumed that certain environmental and genetic factors may contribute to its occurrence.
In our country, although there are no reliable data, the Argentine Association of Respiratory Medicine estimates that the number of cases will be about 8,000, with 2,000 to 3,000 new cases per year. IPF is a disease that typically occurs in people over 60 years of age, and the number of diagnosed people will continue to rise due to increased life expectancy and earlier and more accurate diagnosis due to improved diagnostic methods.
Although in most cases the causes are unknown, there are factors associated with it and they are: smoking, long-term exposure to environmental or occupational pollutants or dust, viral or bacterial lung infections, certain medications such as certain antibiotics, antiarrhythmics, anticonvulsants . ; gastroesophageal reflux disease (GERD); and genetic predisposition
What are the symptoms?
The most common symptom of IPF is shortness of breath (shortness of breath), which many patients describe as a “suffocating feeling”. Other common symptoms are a frequent, irritating dry cough, fatigue and weakness, chest discomfort, loss of appetite, and unexplained weight loss. Symptoms may be absent in the early stages of the disease and appear when it is already running.
The Argentine Association of Respiratory Medicine recommends that people over 60 years of age with symptoms of respiratory distress in the early stages should immediately consult a pulmonologist to exclude this pathology or diagnose it to improve prognosis.
How is it diagnosed?
The diagnosis is made on the basis of a combination of clinical findings, physical examination and additional studies, among which high-resolution CT (computed tomography) is of paramount importance.
Perhaps the biggest challenge is early diagnosis. Clinical manifestations can be common to many respiratory diseases, so it is important to identify patients with risk factors for developing this disease. One option would be to take advantage of the new lung cancer screening strategy, which uses a low-radiation CT scan to look for interstitial changes, perhaps so we can make a diagnosis sooner.
What is the treatment?
Routine medical care may include medication, respiratory rehabilitation, preventive vaccinations, and in some cases supplemental oxygen or lung transplantation.
It is important to highlight the role of pulmonary rehabilitation in improving exercise tolerance and quality of life in these patients, ideally at the time of diagnosis, as well as smoking cessation and prevention of infectious diseases through influenza and pneumococcal vaccination. Two antifibrotic drugs (pirfenidone and nintedanib) are currently available and have been shown to slow the progression of the disease.
In patients who do not respond adequately to treatment, the possibility of lung transplantation remains, with increasingly better results.
They advised: Dr. Viviana Moyano, Erica Cuestas and Lujan Rolando from the Interstitial Diseases Section of the Argentine Association of Respiratory Medicine.