Interstitial lung fibrosis is also known as diffuse interstitial pulmonary fibrosis, and if it’s chronic primary we call it a Hamman-Rich’s syndrome. The ability of the lungs as an organ for exchange of gases is very weakened due to the accumulation of fibrous material which blocks the bronchioles and thickens the walls of the alveoli. Fiber stiffens the lungs and affects their expansion and contraction.
Interstitial lung fibrosis can be acute or chronic. In the acute form, the main symptom is shortness of breath which stronger over time. This symptom occurs after the straining, but as the disease progresses, it may be present even when body is inactive. Cough also occur; it may be without sputum or with bloody sputum. Pain in the chest may be present, too. Disease progression is so fast that most patients die within a year.
The chronic form, which is much more common, starts in middle age, and symptoms, among which the shortness of breath is most important, develop more slowly. Additional symptom of interstitial pulmonary fibrosis is malformation of fingertips – so called digitus fingers – which occurs in other chronic pulmonary diseases, too. The appearance of these symptoms does not mean the disease is in an advanced stage, because digitus fingers may appear surprisingly early.
The exact cause of this rare, severe disease is unknown, but in the UK for the disease accounts for only one of the 100 000 deaths.
Both acute and chronic forms of fibrosis ends in respiratory failure. However, corticosteroids treatment relieves the symptoms and slow down the progression of the disease.