General Information About Idiopathic Pulmonary Fibrosis
Disease Details and Frequently Asked Questions
It is the process by which the lung tissue, which is supposed to stretch like a soft sponge, covers itself with a thick scab (fibrotic tissue), solidifies, and loses its ability to breathe for unknown reasons (idiopathic).
In the idiopathic form (IPF), no definitive cause can be found. However, genetic predisposition, advanced age, and especially SMOKING for many years are the biggest triggers. (If the cause of hardening is inhaling asbestos, silica, or coal dust, it is not IPF but 'Pneumoconiosis/Occupational Disease'. Diseases like Rheumatoid Arthritis can also cause fibrosis).
The disease develops very insidiously over the years. It has two main symptoms: 1) A persistent, dry, hacking COUGH without phlegm. 2) SHORTNESS OF BREATH (Air Hunger) that initially starts when climbing stairs or slopes, but eventually occurs even while getting dressed.
Because the lungs do not send enough oxygen to the body, over the years, the tips of the patient's fingers and toes (nail beds) widen and become domed or drumstick-shaped. This is the most obvious outward manifestation of chronic oxygen deprivation.
When a pulmonologist listens to your back, they will hear a very typical crackling sound every time the stiffened lungs open up, just like the sound of Velcro being pulled apart (Velcro crackles). The definitive diagnosis is made using a High-Resolution Computed Tomography (HRCT) of the lungs. The CT clearly shows the outer parts of the lung taking on a 'Honeycomb' appearance.
Fibrosis (scar tissue) is Permanent; no medication or cortisone can RETURN a stiffened lung to its former soft state. If untreated, the disease is fatal within 3-5 years.
However, thanks to the medical revolution of Anti-fibrotic drugs (Pirfenidone and Nintedanib) today, the scar formation process in the lung is largely stopped or significantly slowed down. Patients are given an Oxygen Machine (Concentrator) for lifelong use. For end-stage patients under 65 where medications are insufficient, the only definitive solution is a Lung Transplant.
Our health library contents are prepared for informational purposes only and with scientific data available at the time of recording. For all your questions, concerns, diagnosis, or treatment regarding your health, please consult your doctor or a healthcare institution.
