Pulmonary fibrosis is a serious pathological condition that affects lung tissue, causing the progressive replacement of normal parenchyma (i.e. the functional tissue of the lung) with fibrous tissue. This leads to lung stiffening and impaired gas exchange, making breathing increasingly difficult. Globally, idiopathic pulmonary fibrosis (IPF)—the most severe and widely studied form of fibrosis—is considered a rare disease with a growing impact, particularly in elderly populations.
According to a meta-analysis that collected data from observational studies published between 2000 and 2023, there are approximately 5.8 new IPF diagnoses per 100,000 people per year worldwide, and approximately 17.7 people per 100,000 are living with the disease globally. These figures confirm that IPF is a rare but not exceptional condition, with higher numbers in North America than in Europe and Asia.
A recent study conducted by researchers in Tuscany (Italy) confirms these global figures, estimating that approximately 21.5 people per 100,000 in Tuscany are living with a diagnosis of idiopathic pulmonary fibrosis (IPF), with an incidence rate of 4.6 new cases per 100,000 people per year, and a five-year survival rate below 55% after diagnosis.
Other observational studies by Chinese researchers suggest that, despite advances in the treatment of pulmonary fibrosis, life expectancy remains reduced, with an average survival of 2–5 years after diagnosis in the absence of appropriate treatment. This prognosis varies according to age, gender, and stage of the disease.
But what does living with this condition really mean? To find out, we spoke to Dr Josuel Ora, a pulmonologist and lecturer in respiratory diseases at UniCamillus University, who helps put these statistics into clinical context for patients.
What is pulmonary fibrosis, and what causes it?
Pulmonary fibrosis is part of a broad group of over 200 conditions known as interstitial lung diseases (ILDs), which share a similar final pathogenic mechanism. “Normal lung tissue is progressively replaced by fibrous tissue, which is a kind of scar tissue that makes the lung stiffer and less efficient at exchanging oxygen”, explains Dr Ora. Despite this common feature, some forms of ILD have very different characteristics and clinical courses.
Pulmonary fibrosis can be caused by environmental or occupational exposure to mineral, organic or metallic dust, welding fumes, or chemical vapours; autoimmune diseases; drug treatments; radiotherapy; or chronic inflammatory processes. However, in many cases, it is not possible to identify a specific cause. In these situations, we refer to idiopathic pulmonary fibrosis”, explains Dr Ora. “Idiopathic pulmonary fibrosis is one of the main forms of interstitial lung disease and is characterised by a progressive decline in respiratory function. Unlike other interstitial diseases with a marked inflammatory component, IPF is dominated by irreversible fibrotic damage”.
Correctly identifying the origin of fibrosis is essential for choosing the right treatment and defining life expectancy.
Symptoms are often underestimated and the disease progresses silently
The initial symptoms of pulmonary fibrosis are often vague and develop slowly over time. The most common symptoms are a chronic dry cough and shortness of breath during exertion. These symptoms are easily underestimated because they are often attributed to trivial causes. “A dry cough is often considered normal, especially in smokers”, notes Dr Ora, highlighting how this can mislead people into not seeking immediate medical advice. In addition, many patients unknowingly change their habits to compensate for fatigue, thus delaying diagnosis. They gradually reduce their level of physical activity and avoid strenuous exertion, eventually no longer perceiving the change as a real symptom of disease but rather as a normal consequence of ageing or lifestyle. “Paradoxically, athletes are often the first to notice a drop in performance, as they are more attuned to physical effort and immediately recognise when activities that were once effortless become unexpectedly tiring”.
This delay in diagnosis is particularly critical because pulmonary fibrosis is progressive: intervention in the early stages can slow progression and preserve respiratory function for longer. As the condition worsens, the lung tissue becomes increasingly fibrotic and rigid, forming actual scars. Unlike fibrotic sequelae, these active scars drive the disease forward, resulting in a decrease in respiratory volume and worsening gas exchange.
“In the early stages, oxygen levels drop during exertion. In the more advanced stages, this can lead to respiratory failure even at rest”, explains Dr Ora. “These pulmonary scars profoundly affect quality of life. Exercise tolerance is reduced, meaning the person is able to perform fewer and fewer activities, and over time they may require oxygen therapy during exertion. Oxygen therapy delivers more oxygen to the blood when the lungs are unable to do so on their own”. However, Dr Ora also emphasises how difficult it is to incorporate oxygen therapy into daily life. “Some patients refuse it and progressively reduce the time they spend outside the home, further limiting their social interactions. As the condition worsens, hospitalisations may increase, as may cardiac complications, because the heart and lungs share space and circulation within the chest”.
Diagnosis: radiology and spirometry
How is interstitial fibrosis diagnosed? The diagnostic process begins with clinical suspicion based on the patient’s medical history and a physical examination. Auscultation may reveal dry crackles in the lower parts of the lungs, known as ‘Velcro-like’ crackles because they resemble the sound of crumpled Velcro.
“High-resolution chest CT scans can confirm fibrosis and define its radiological pattern. By integrating radiology, laboratory tests and clinical history, we can diagnose idiopathic pulmonary fibrosis or other forms of fibrosis”, says our expert, explaining that spirometry and respiratory function tests are essential for monitoring disease progression. “These tests provide the best window into respiratory mechanics and gas exchange, both at rest and during exertion, and they can be repeated over time”.
Available therapies
There are currently two approved treatments for idiopathic pulmonary fibrosis: pirfenidone and nintedanib. “These drugs do not reverse the fibrotic process, but they do slow its progression. This results in improved survival, even though existing damage is not repaired and symptoms persist”. Nintedanib can also benefit patients with non-idiopathic forms of progressive pulmonary fibrosis.
Dr Ora emphasises the importance of respiratory rehabilitation in the treatment plan. “A recent meta-analysis shows that it improves physical activity in moderate cases and produces positive effects even in more advanced stages.” Rehabilitation must, of course, be combined with drug therapies and oxygen therapy when necessary.
Prognosis and life expectancy
According to the expert, the prognosis for idiopathic pulmonary fibrosis is generally poor, although it has improved in recent years thanks to medication. Other types of pulmonary fibrosis may have better prognoses. Predictions are difficult to make; what matters is how the disease progresses in each individual patient.
“Firstly, it is important not to face this situation alone, but to rely on expert centres and professionals. It is important to remain as active as possible, attend therapies and check-ups, and to pay attention to their psychological well-being”, concludes Dr Ora.
Accepting the disease does not mean giving up; it means finding a new balance and not letting difficult moments cause despair or disrupt your social life. With the right strategies, it is entirely possible to maintain a good quality of life.
Dr Josuel Ora teaches third-year medical students.