Also known as: Pulmonary Fibrosis / Idiopathic Pulmonary Fibrosis (IPF)
Pulmonary fibrosis is a progressive lung disease in which lung tissue becomes scarred and thickened, making it increasingly difficult to breathe. In many cases, the cause is unknown (idiopathic).
Pulmonary fibrosis encompasses a group of diseases in which the lung interstitium becomes progressively scarred. Idiopathic pulmonary fibrosis (IPF) is the most common and severe form, affecting approximately 13–20 per 100,000 people.
The scarring thickens the tissue around and between the air sacs, making it progressively harder for oxygen to pass into the bloodstream. The condition is irreversible — once scar tissue forms, it cannot be removed.
Early diagnosis is important as antifibrotic medications can slow the rate of decline. Lung transplantation may be considered for eligible patients with advanced disease.
People with Pulmonary Fibrosis often experience the following symptoms.
Shortness of breath that initially occurs only with exertion but gradually worsens to affect even routine activities and eventually rest.
A persistent, non-productive cough that can be debilitating and resistant to standard cough treatments.
Enlargement and rounding of the fingertips and nails, occurring in approximately 25–50% of IPF patients.
Fine, dry crackles heard through a stethoscope at the lung bases, resembling the sound of pulling apart Velcro. A characteristic early finding.
Certain factors may increase your likelihood of developing Pulmonary Fibrosis.
Common approaches to managing pulmonary fibrosis. Always consult a healthcare provider for personalized treatment.
Pirfenidone and nintedanib are the two approved antifibrotic drugs that slow the rate of lung function decline in IPF.
Supervised exercise programmes, breathing techniques, and education improve quality of life and exercise tolerance.
Prescribed when blood oxygen levels drop, initially with exertion and eventually at rest as the disease progresses.
May be considered for eligible patients with advanced disease. It is the only treatment that can substantially extend survival in severe cases.
Diagnosis involves high-resolution CT scan (showing a characteristic 'usual interstitial pneumonia' pattern), pulmonary function tests (showing restrictive pattern), and clinical evaluation. Surgical lung biopsy may be needed if imaging is inconclusive. Blood tests help exclude autoimmune causes.
See a doctor promptly if you experience worsening shortness of breath, persistent dry cough, or if you notice a decline in your exercise tolerance over weeks to months.
Steps that may help reduce the risk of developing or worsening pulmonary fibrosis.
Avoid smoking and secondhand smoke
Use proper respiratory protection in dusty environments
Follow safety guidelines when handling asbestos or chemicals
Treat GERD, which may contribute to lung injury
If left untreated or poorly managed, pulmonary fibrosis may lead to:
Unfortunately, existing scar tissue cannot be reversed. However, antifibrotic medications can significantly slow further scarring, and pulmonary rehabilitation can improve quality of life.
Average survival from diagnosis is 3–5 years, but there is significant variability. Some patients remain stable for years while others decline more rapidly. Early treatment improves outcomes.
No. Both cause breathing difficulty but through different mechanisms. COPD involves airway obstruction and air trapping, while pulmonary fibrosis involves scarring that restricts lung expansion. They require different treatments.
This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.