Tuesday, 14. November 2023

Idiopathic pulmonary fibrosis: When the lungs are scarred

Idiopathic pulmonary fibrosis causes the lung tissue to continuously scar. There are milder courses of the disease with a gradual deterioration or more severe courses with episodes of deterioration. What does a diagnosis mean for patients and how does idiopathic pulmonary fibrosis develop? We have outlined the critical points for you.

What does idiopathic pulmonary fibrosis mean?

Elderly man standing on stairs with his hand on his lungs, finding it difficult to breathe — Idiopathic pulmonary fibrosis: No room to breathe

Idiopathic pulmonary fibrosis is abbreviated as IPF. The word “fibrosis” is used to describe pathological changes to the structure of the organ tissue. In medicine, “idiopathic” means any disease whose exact cause has yet to be identified. IPF, therefore, means pathological scarring of the lung tissue without a clear cause.

It is also considered a rare disease, such as cystic fibrosis (CF). Unlike IPF, however, the causes of CF are known. The exact figures for idiopathic pulmonary fibrosis are not known. It is, however, assumed that in Europe about 5 in 100,000 people are affected. It is difficult to diagnose IPF. This is because the symptoms are often similar to the symptoms of other diseases, such as COPD or asthma.

What happens to the lungs of a patient with idiopathic pulmonary fibrosis?

Essentially, scarring is the body’s natural response to injury. However, in IPF, the normal healing process is impaired and excessive scar tissue develops in the lungs. This involves a massive increase in the connective tissue cells (fibroblasts). They bind together to the size of a pin head, known as a “fibroblast foci”. These lesions are characteristics of idiopathic pulmonary fibrosis.

The fibroblasts form the diseased scar tissue, which little by little displaces the healthy alveoli of the lungs. The scar tissue in the lungs ends up looking like a honeycomb, hence the process is called “honeycombing”. Patients ultimately suffocate once the scar tissue has destroyed most of the alveoli. This is why a lung transplant is often the last available option in the most serious cases.

What do we know about how idiopathic pulmonary fibrosis develops?

Elderly man sits with his hand on his chest in front of the doctor, who explains something to him — Idiopathic pulmonary fibrosis: Diagnosis

Severe exposure of the lungs to harmful substances can increase the risk of developing idiopathic pulmonary fibrosis. These include, for instance, cigarette smoke or dust from metal or wood. There have also been investigations into whether there may be a link to a prior infection. However, no evidence has yet been uncovered that this may cause IPF.

Genetic factors are also being explored but have so far failed to yield any evidence. Reflux or heartburn is also suspected to be associated with idiopathic pulmonary fibrosis, as this can lead to tiny acid burns in the lungs, which in turn can impair the healing process.

While all these factors may increase the risk of developing IPF, the cause of the disease has not yet been identified.