Pulmonary Fibrosis: Causes, Symptoms and Treatment
Discover the causes, symptoms, diagnosis, and treatment options for pulmonary fibrosis. Learn how to manage this progressive lung disease and improve quality of life.
Written by Dr.Sonia Bhatt
Last updated on 3rd Jul, 2025
Introduction
Pulmonary fibrosis refers to a group of serious lung diseases that affect the respiratory system, causing scarring and thickening of lung tissue. It primarily impacts the connective tissue in the lungs and the alveoli (the tiny air sacs responsible for oxygen exchange).
Over time, lung damage worsens, making the tissues hard and stiff, which reduces their ability to expand properly. As a result, breathing becomes more difficult, and even routine activities may cause breathlessness. In this blog, we will explore pulmonary fibrosis in detail.
Causes of Pulmonary Fibrosis
The common causes of pulmonary fibrosis are as follows:
Lung Tissue Scarring – Thickening and scarring of the tissue surrounding the alveoli (air sacs) in the lungs, making oxygen transfer to the bloodstream more difficult.
Exposure to Toxins – Long-term inhalation of harmful substances such as silica dust, asbestos, coal dust, or farming chemicals.
Radiation Therapy – Previous radiation treatment to the chest, particularly for cancer, may lead to lung damage.
Certain Medications – Some drugs, including chemotherapy agents, heart medications, and antibiotics, can contribute to lung scarring.
Underlying Medical Conditions – Autoimmune diseases such as rheumatoid arthritis, scleroderma, and lupus may increase the risk of pulmonary fibrosis.
Idiopathic Pulmonary Fibrosis (IPF) – In many cases, the exact cause remains unknown, and the condition is classified as idiopathic.
Pathophysiology of Pulmonary Fibrosis
Pulmonary fibrosis develops through a complex process of lung injury, inflammation, and excessive tissue scarring, leading to progressive impairment of lung function.
Injury – Pulmonary fibrosis can result from various factors, including environmental pollutants, infections, autoimmune or allergic reactions, or mechanical damage.
Inflammation – The initial injury triggers inflammation, leading to cellular damage and tissue destruction.
Fibroblast Proliferation – Damaged tissue and immune cells release cytokines, stimulating the proliferation of fibroblasts.
Myofibroblast Formation – Fibroblasts differentiate into myofibroblasts, which produce fibrous proteins and ground substance, forming the extracellular matrix (ECM).
Thickening and Fibrosis – The accumulation of ECM causes lung tissue to become thickened and fibrotic, resulting in irreversible scarring that impairs gas exchange and lung function.
Symptoms of Pulmonary Fibrosis
The symptoms of pulmonary fibrosis can vary in severity and progression from person to person. Common symptoms include:
Shortness of breath
Persistent dry cough
Severe fatigue
Unexplained weight loss
Muscle and joint pain
Clubbing – widening and rounding of the fingertips or toes
The rate at which pulmonary fibrosis progresses differs among individuals. Some may develop severe symptoms rapidly, while others experience a gradual decline over months or years.
Risk Factors for Pulmonary Fibrosis
Although pulmonary fibrosis can occur in infants and children, it is uncommon. Idiopathic pulmonary fibrosis is more frequently seen in middle-aged and older adults, while other forms, such as those linked to connective tissue diseases, may affect younger individuals.
Several factors can increase the risk of developing pulmonary fibrosis, including:
Smoking – Current and former smokers are at greater risk, particularly those with emphysema.
Occupational exposure – People working in industries such as mining, farming, and construction face a higher risk due to prolonged exposure to lung-damaging pollutants.
Cancer treatments – Radiation therapy to the chest and certain chemotherapy drugs may contribute to the development of pulmonary fibrosis.
Genetics – Some forms of pulmonary fibrosis run in families, suggesting a genetic predisposition.
Diagnosis of Pulmonary Fibrosis
Diagnosing pulmonary fibrosis involves a thorough assessment of medical and family history, a physical examination, and various diagnostic tests. Your healthcare professional will discuss your symptoms, medication history, and potential exposure to harmful substances, such as dust, chemicals, or gases.
During the physical examination, the doctor will listen to your lungs for abnormal crackling sounds, which are often associated with pulmonary fibrosis. The following tests may be performed to confirm the diagnosis and assess lung function:
1. Imaging and Pulmonary Function Tests
The imaging and pulmonary function tests are as follows:
1. Chest X-ray – May reveal lung scarring, though further tests may be required if the X-ray appears normal.
2. High-Resolution CT (HRCT) Scan – Produces detailed images of the lungs to detect fibrosis patterns and assess lung damage.
3. Echocardiogram – Uses ultrasound to evaluate heart function and measure pressure in the lung arteries.
4. Pulmonary Function Tests (PFTs) – These assess lung capacity and efficiency, including:
Spirometry – Measures how much air the lungs can hold and how forcefully air can be exhaled.
Lung Volume Test – Evaluates the total capacity of the lungs.
Lung Diffusion Test – Assesses the lungs' ability to transfer oxygen into the blood.
5. Pulse Oximetry – A non-invasive test that measures blood oxygen levels using a fingertip sensor.
6. Exercise Stress Test – Evaluates lung and heart function during physical activity.
7. Arterial Blood Gas Test – Analyses blood samples to determine oxygen and carbon dioxide levels.
2. Biopsy and Histopathology
If imaging and lung function tests are inconclusive, a lung biopsy may be necessary to confirm pulmonary fibrosis and rule out other conditions. Biopsy methods include:
Surgical Biopsy – A sample of lung tissue is removed through a procedure such as:
Video-Assisted Thoracoscopic Surgery (VATS) – A minimally invasive procedure using small incisions and a camera-guided approach.
Thoracotomy – An open-chest procedure for obtaining larger tissue samples.
Bronchoscopy – A flexible tube is passed through the nose or mouth into the lungs to extract small tissue samples. While less invasive, these samples may sometimes be too small for an accurate diagnosis.
Blood Tests – Used to assess liver and kidney function and rule out other possible conditions contributing to lung disease.
Treatment Options for Pulmonary Fibrosis
Pulmonary fibrosis is a progressive condition with no known cure. While current treatments cannot reverse lung scarring, they may help manage symptoms, slow disease progression, and improve quality of life. Treatment plans are tailored based on the severity of the condition and individual patient needs.
1. Medications and Therapies
Although no medication can fully stop pulmonary fibrosis from worsening, some medicines may help slow disease progression and manage symptoms:
Antifibrotic Medicines –
Pirfenidone (Esbriet) and Nintedanib (Ofev) are approved to slow the progression of idiopathic pulmonary fibrosis (IPF). Nintedanib is also used for other rapidly worsening types of pulmonary fibrosis.
These medicines may cause side effects such as nausea, loss of appetite, skin sensitivity, or diarrhoea. Regular liver function tests may be needed.
Gastroesophageal Reflux Disease (GERD) Treatment – Many people with IPF also experience GERD, so anti-acid medications may be prescribed to manage acid reflux.
Clinical Trials and Emerging Therapies – Ongoing research is exploring new treatments for pulmonary fibrosis, offering potential future options.
2. Oxygen Therapy and Supportive Care
Oxygen therapy does not reverse lung damage but can help improve breathing, reduce complications, and enhance quality of life.
Supplemental Oxygen – Used during activity, sleep, or continuously, depending on individual needs. Portable oxygen tanks or concentrators can support mobility.
Pulmonary Rehabilitation – A structured programme designed to enhance lung function and overall well-being, including:
Physical exercises to improve endurance.
Breathing techniques to optimise oxygen use.
Nutritional guidance.
Emotional support and counselling.
Education on managing symptoms.
Managing Acute Exacerbations – Sudden worsening of symptoms may require hospitalisation, increased oxygen support, or mechanical ventilation. Treatment may include antibiotics, corticosteroids, or other medications.
3. Lung Transplantation
For individuals with severe pulmonary fibrosis, a lung transplant may be a viable treatment option.
Benefits – Can significantly improve breathing, quality of life, and life expectancy.
Risks – Includes potential complications such as organ rejection and infections. Lifelong immunosuppressive medications are required post-transplant.
Eligibility – Not all patients qualify for a lung transplant. Decisions are based on overall health, age, and disease severity.
Lifestyle and Management
Making key lifestyle changes can help manage pulmonary fibrosis and improve well-being.
Quit Smoking – Seek support to stop smoking and avoid second-hand smoke.
Limit Lung Irritants – Reduce exposure to indoor fumes, chemicals, dust, and outdoor pollutants.
Eat Well – Maintain a balanced diet with sufficient calories; eat smaller, frequent meals if needed.
Stay Active – Engage in suitable exercises like walking or tai chi; consult your healthcare team.
Rest Adequately – Prioritise sleep and manage stress for better energy levels.
Get Vaccinated – Keep up with flu, pneumonia, and COVID-19 vaccines to prevent infections.
Follow Treatment – Adhere to medications, oxygen therapy, and pulmonary rehabilitation.
Conclusion
Pulmonary fibrosis is a progressive lung disease that requires careful management to maintain quality of life. While there is no cure, early diagnosis, appropriate treatment, and lifestyle adjustments can help slow disease progression and ease symptoms. Staying proactive with medical care, avoiding lung irritants, and maintaining a healthy lifestyle are key to managing the condition. With the right approach, individuals with pulmonary fibrosis can improve their well-being and live as actively as possible.
Consult Top Respiratory Disease Specialist
Consult Top Respiratory Disease Specialist
Dr Abhishek Verma
Pulmonology Respiratory Medicine Specialist
5 Years • MD (Respiratory Medicine), PDCC (Interventional Pulmonology) Alumni SGPGIMS & KGMU Lucknow
Lucknow
Apollomedics Super Speciality Hospital, Lucknow
(50+ Patients)
Dr. S Mallikarjun Rao
Pulmonology Respiratory Medicine Specialist
22 Years • MBBS, MD (Pul.), FCCP
Hyderguda
Apollo Hospitals Hyderguda, Hyderguda
(75+ Patients)
Dr. Krishna Ramanathan
Ent Specialist
10 Years • MBBS DNB
Bengaluru
Apollo Clinic, JP nagar, Bengaluru
Dr Haripriya S G
Family Physician
22 Years • MBBS, PGD (Family Medicine)
Bengaluru
Apollo Clinic, JP nagar, Bengaluru
Dr. Raj Kumar
Pulmonology Respiratory Medicine Specialist
20 Years • MBBS, MD (Tuberculosis & Chest Disease), FCICM ( Critical Care Australia)
Delhi
Hope for Lungs, Delhi
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