Progressive Fibrosing
By Progressive we mean
- the condition gets worst over time
- there is a worsening of respiratory symptoms (e.g. increasing shortness of breath)
- the lung function declines rapidly and the scar tissue keeps increasing, even when the patient is being treated with corticosteroids or other immunosuppressant drugs.
Progression is disease-specific, varies from person to person and is reflected in a decline in lung function, worsening of symptoms and deterioration in health-related quality of life. It is impossible to predict exactly how rapidly fILD will progress for an individual. Many people with fILD experience a slow, but steady worsening of their disease. If fILD is progressing slowly, patients could have the symptoms for a long time before being diagnosed, and then the disease might still progress relatively slowly. While IPF is almost always progressive, in other forms of fILD similar progression is seen in about one third of patients.
Some patients may experience unpredictable acute worsening of their disease, called acute exacerbations, which can happen at any time. An event like this may be fatal or may leave a patient with substantially worsened disease. Sometimes a person is not diagnosed with fILD until their first acute exacerbation. The main risk factor for acute exacerbation of fILD is advanced disease.
Other conditions patients with fILD might have (comorbidities)
FILD can be a debilitating condition as on top of the adverse effects of fILD, most fILD patients have other associated conditions (comorbidities). They can have a negative effect on quality of life and prognosis. This is why doctors also need to identify and treat any comorbidities patients may develop.
These conditions can affect the lungs (pulmonary comorbidities like lung cancer and emphysema) or other parts of the body (nonpulmonary comorbidities like gastroesophageal reflux disease and cardiovascular diseases).
The danger of an acute exacerbation makes regular monitoring by professionals essential so they can track how the disease is progressing and see how a patient is responding to treatment.
By Fibrosing we mean
- presence of pulmonary fibrosis (scarring in the lungs) detected by an HRCT* (a scan of the chest to look at the lung in detail)
- the scar tissue builds up in the walls of the air sacs of the lungs and eventually makes it hard for oxygen to get into the blood
- low oxygen levels (and the stiff scar tissue itself) can cause shortness of breath, particularly when walking and exercising