Pulmonary Fibrosis Treatment in Delhi, India

Pulmonary fibrosis is a progressive lung disease characterised by the formation of scar tissue (fibrosis) in the lungs. This scarring thickens and stiffens the lung tissue, making it difficult for the lungs to function properly. Over time, pulmonary fibrosis can lead to symptoms such as shortness of breath, coughing, and fatigue. While there is no cure for pulmonary fibrosis, there are treatments that can help slow the progression of the disease and manage symptoms.

Pulmonary fibrosis can be classified into several types. Here are the main types and their causes.

• Asbestosis: It is a type of pulmonary fibrosis caused by prolonged exposure to asbestos fibres, commonly found in occupations such as mining, construction, or shipbuilding.
• COVID-19-related pulmonary fibrosis: Another pulmonary fibrosis type that is related to COVID-19 and results from severe infection, leading to scarring in the lungs as a long-term complication.
• Drug-induced pulmonary fibrosis: Pulmonary fibrosis induced by drugs can occur due to certain medications, including chemotherapy drugs, antibiotics like nitrofurantoin or amiodarone, and some biological agents.
• Hypersensitivity Pneumonitis (HP): HP is triggered by inhaling allergens or irritants such as mould, animal dander, or chemicals, which provoke an inflammatory response in the lungs.
• Idiopathic pulmonary fibrosis (IPF): IPF's cause is unknown but is believed to involve genetic predisposition, environmental factors, and abnormal wound healing processes in the lungs.
• Idiopathic NSIP (Non-Specific Interstitial Pneumonitis): The cause of NSIP is unknown, but it presents with a more uniform pattern of inflammation and fibrosis in the lungs compared to IPF.
• Pneumoconiosis: A common pulmonary fibrosis type, Pneumoconiosis, results from inhaling mineral dust particles, including coal dust (coal workers' pneumoconiosis), silica dust (silicosis), or asbestos fibres (asbestosis), typically in occupational settings.
• Rheumatoid Arthritis Interstitial Lung Disease (RA-ILD): It is a complication of rheumatoid arthritis, where inflammation spreads to the lungs, leading to fibrosis and impaired lung function.
• Sarcoidosis: Sarcoidosis's cause is unknown, but it is thought to involve an abnormal immune response, resulting in the formation of granulomas (inflammatory nodules) in various organs, including the lungs.
• Silicosis: Silicosis occurs due to inhaling silica dust, commonly found in industries such as mining, construction, or sandblasting where silica-containing materials are processed.
• Systemic sclerosis: Systemic sclerosis is an autoimmune disease affecting connective tissue, leading to fibrosis and thickening of the skin and internal organs, including the lungs.

Factors that contribute to increased susceptibility to pulmonary fibrosis include:

• Age: While pulmonary fibrosis can affect individuals of all ages, it is more commonly diagnosed in middle-aged and older adults.
• Sex: Idiopathic pulmonary fibrosis tends to affect men more frequently than women.
• Smoking: Individuals who smoke or have a history of smoking are at a higher risk of developing pulmonary fibrosis, particularly those with emphysema.
• Occupational exposure: Certain occupations, such as mining, farming, or construction, pose an increased risk of pulmonary fibrosis due to exposure to lung-damaging pollutants.
• Cancer treatments: Undergoing radiation therapy to the chest or receiving specific chemotherapy drugs can elevate the risk of developing pulmonary fibrosis.
• Genetic predisposition: Certain types of pulmonary fibrosis have a familial pattern, suggesting a genetic component in their development.

Pulmonary fibrosis symptoms can vary from person to person and may develop gradually or suddenly. Common symptoms include:

• Loss of appetite
• Persistent dry cough
• Fatigue or weakness
• Gradual unintended weight loss
• Aching muscles and joints
• Chest discomfort or tightness
• Shortness of breath, especially during physical activity
• Widening and rounding of the tips of the fingers or toes, known as clubbing

It's important to note that these symptoms can resemble those of other lung conditions, so it's crucial to consult a healthcare professional for proper diagnosis and treatment if someone experiences any of these symptoms. Early detection and intervention can help manage symptoms and improve quality of life for individuals with pulmonary fibrosis.

Upon suspicion of pulmonary fibrosis, a comprehensive diagnostic approach is crucial for accurate assessment and effective management. Here are some of the tests a doctor may recommend besides the regular physical examination.

Imaging tests

• Chest X-Ray: A commonly used initial imaging test that provides a basic view of the lungs. It can show signs of scarring or fibrosis.
• Computerised Tomography (CT) scan: Provides more detailed images of the lungs than X-rays, allowing for better visualisation of lung tissues and abnormalities such as fibrosis.
• Echocardiogram: While primarily used to evaluate the heart, an echocardiogram can also detect changes in the heart that may be associated with pulmonary fibrosis, such as pulmonary hypertension.

Lung function tests

• Pulmonary Function Test (PFT): Assesses lung function by measuring how much air you can inhale and exhale, how quickly you can exhale, and how efficiently gases are exchanged.
• Pulse oximetry: Measures the oxygen saturation of the blood, providing information about how well the lungs are oxygenating the blood.
• Exercise stress test: Evaluates how well the lungs function under stress, such as during physical activity, by measuring breathing and heart rate responses to exercise.
• Arterial blood gas test: Measures the levels of oxygen and carbon dioxide in the blood, providing information about lung function and gas exchange.

Biopsy

• Bronchoscopy: A procedure in which a thin, flexible tube with a camera (bronchoscope) is inserted through the mouth or nose into the airways to collect small samples of lung tissue for examination.
• Surgical biopsy: Involves the removal of a larger piece of lung tissue through a surgical procedure, typically when bronchoscopy does not provide enough tissue for diagnosis.

Blood tests

Blood tests may be performed to assess overall health and check for conditions that could contribute to pulmonary fibrosis, such as autoimmune diseases or infections. These tests may include measures of inflammation, immune function, and blood cell counts.

These tests are used in combination to diagnose pulmonary fibrosis, assess its severity, and monitor disease progression. Depending on the individual case and the suspected cause of pulmonary fibrosis, additional tests or imaging studies may be necessary.

Treatment for pulmonary fibrosis aims to manage symptoms, improve quality of life, and slow disease progression. While lung scarring cannot be reversed, some treatments may provide temporary relief or slow down the advancement of the condition. Here is an overview of the various treatment options available:

Medications

• Pirfenidone (Esbriet) and Nintedanib (Ofev): These newer medications have shown promise in slowing the progression of idiopathic pulmonary fibrosis (IPF). Approved by the FDA, they work by targeting pathways involved in fibrosis. However, they may cause side effects such as diarrhoea, nausea, and rash. Research is ongoing to develop additional medications and formulations to further improve treatment outcomes.
• Anti-acid medications: Gastroesophageal reflux disease (GERD) commonly occurs in people with IPF. Anti-acid medications may be prescribed to manage GERD symptoms and potentially reduce lung inflammation.

Oxygen therapy

While oxygen therapy cannot reverse lung damage, it can improve symptoms and quality of life by making breathing and exercise easier, preventing complications from low blood oxygen levels, and reducing pulmonary hypertension. Oxygen may be used during sleep, exercise, or continuously, depending on individual needs.

Pulmonary Rehabilitation

Pulmonary rehabilitation programs focus on enhancing physical endurance, teaching breathing techniques to optimise lung function, providing nutritional counselling, offering emotional support and education about the disease. These programs aim to improve daily functioning and overall well-being.

Lung Transplant

For some individuals with advanced pulmonary fibrosis, lung transplantation may be considered. A lung transplant can significantly improve quality of life and prolong survival. However, it carries risks such as rejection and infection. Candidates for lung transplantation undergo thorough evaluation to determine suitability for the procedure.

At Max Hospitals, Delhi, we offer personalised pulmonary fibrosis treatments based on the severity of the condition, individual symptoms, and patient preferences.

Pulmonary fibrosis can lead to several complications, which can significantly impact a person's health and quality of life. Some common complications associated with pulmonary fibrosis include:

• Respiratory failure: As the lung tissue becomes increasingly scarred and stiff, it can impair the lungs' ability to transfer oxygen into the bloodstream, leading to respiratory failure. This can result in severe difficulty breathing and require supplemental oxygen or mechanical ventilation.
• Pulmonary hypertension: Pulmonary fibrosis can cause increased pressure in the pulmonary arteries, known as pulmonary hypertension. This can strain the heart and lead to symptoms such as shortness of breath, fatigue, and chest pain.
• Collapsed lung (Pneumothorax): In some cases, the formation of bullae (large air-filled spaces) within the lungs can lead to a collapsed lung, known as a Collapsed lung (Pneumothorax). This can cause sudden chest pain and difficulty breathing.
• Cor pulmonale: Chronic pulmonary hypertension resulting from pulmonary fibrosis can lead to a condition called cor pulmonale, where the right side of the heart becomes enlarged and weakened. Symptoms may include swelling in the legs and abdomen, fatigue, and difficulty breathing.
• Respiratory infections: Individuals with pulmonary fibrosis are at increased risk of developing respiratory infections, such as pneumonia, due to impaired lung function and compromised immune response.
• Emotional and psychological impact: Living with a chronic and progressive lung disease like pulmonary fibrosis can take a toll on a person's mental health and emotional well-being. Anxiety, depression, and stress are common among individuals with pulmonary fibrosis and can affect their overall quality of life.
• Reduced exercise tolerance: Shortness of breath and decreased lung function can limit physical activity and exercise tolerance, leading to deconditioning and muscle weakness.
• Pulmonary embolism: Individuals with pulmonary fibrosis may be at higher risk of developing blood clots in the pulmonary arteries, known as pulmonary embolism. This can cause sudden onset chest pain, shortness of breath, and can be life-threatening if not treated promptly.
• Complications of treatment: Some medications used to manage pulmonary fibrosis, such as immunosuppressants or antifibrotic drugs, can have side effects and may increase the risk of certain complications.

It's essential for individuals with pulmonary fibrosis to work closely with their healthcare providers to monitor their condition, manage symptoms, and address any complications that may arise. Regular follow-up appointments and adherence to treatment plans are crucial for optimising quality of life and reducing the risk of complications.

To prevent pulmonary fibrosis, it's crucial to take proactive steps to protect your respiratory health and minimise exposure to known risk factors. Here's how you can do it:

• Avoid smoking: Smoking is a significant risk factor for pulmonary fibrosis. If you smoke, quitting is the single most important step you can take to protect your lungs. Avoid exposure to secondhand smoke as well, as it can also contribute to lung damage.
• Reduce exposure to environmental pollutants: Be mindful of your surroundings and minimise exposure to airborne toxins, dust, and chemical fumes, especially in workplaces or industrial settings. Use proper protective equipment and ventilation when working with hazardous materials.
• Practise good respiratory hygiene: Prevent respiratory infections by practising good respiratory hygiene. Cover your mouth and nose when coughing or sneezing, wash your hands frequently, and avoid close contact with sick individuals, especially during flu season.
• Maintain a healthy lifestyle: A balanced diet rich in fruits, vegetables, whole grains, and lean proteins supports overall health and immune function. Regular exercise promotes cardiovascular health and maintains lung function.
• Manage underlying medical conditions: Certain medical conditions, such as gastroesophageal reflux disease (GERD) or autoimmune disorders, can increase the risk of pulmonary fibrosis. Work with your healthcare provider to manage these conditions effectively.
• Follow workplace safety guidelines: If you work in industries with potential exposure to lung irritants or toxins, adhere to safety protocols and use appropriate protective equipment. Participate in workplace safety training to minimise the risk of occupational lung diseases.
• Consider genetic counselling: If there's a family history of pulmonary fibrosis or related lung diseases, genetic counselling can provide valuable insight into potential risks and help you make informed decisions about your respiratory health.

By incorporating these preventive measures into your lifestyle, you can reduce your risk of developing pulmonary fibrosis and safeguard your respiratory well-being for the long term.

Is pulmonary fibrosis a terminal illness?

Pulmonary fibrosis can be a progressive and life-threatening condition, but not all cases are terminal. The prognosis varies depending on factors such as the underlying cause, the severity of lung damage, and the individual's overall health. Some people with pulmonary fibrosis may live for many years with proper management, while others may experience a more rapid decline in lung function.

Are pulmonary fibrosis and COPD same?

No, pulmonary fibrosis and chronic obstructive pulmonary disease (COPD) are not the same. While both are chronic lung diseases, they have different underlying causes and characteristics. Pulmonary fibrosis involves scarring and stiffening of the lung tissue, leading to decreased lung function and difficulty breathing. COPD, on the other hand, primarily involves airflow obstruction due to inflammation and narrowing of the airways, often caused by smoking or environmental factors.

How common is pulmonary fibrosis?

Pulmonary fibrosis is relatively rare compared to other respiratory conditions. It is estimated to affect around 5 million people worldwide. However, the incidence of pulmonary fibrosis appears to be increasing, possibly due to better recognition and diagnosis of the condition.

Who is more likely to get pulmonary fibrosis?

While pulmonary fibrosis can affect people of all ages and backgrounds, it is more common in older adults, particularly those over the age of 50. Men are also slightly more likely than women to develop pulmonary fibrosis. Certain risk factors, such as smoking, exposure to environmental toxins, and a family history of lung disease, may increase the likelihood of developing pulmonary fibrosis.

Can pulmonary fibrosis be hereditary?

In some cases, pulmonary fibrosis can have a genetic component. Familial pulmonary fibrosis refers to cases where multiple family members are affected by the condition. While specific genetic mutations have been identified in some cases of familial pulmonary fibrosis, the inheritance pattern can vary. However, most cases of pulmonary fibrosis are not hereditary.

Can pulmonary fibrosis be reversed?

Pulmonary fibrosis involves irreversible scarring and damage to the lung tissue, so it cannot be fully reversed. However, treatment may help slow the progression of the disease, manage symptoms, and improve quality of life for individuals living with pulmonary fibrosis.

Can pulmonary fibrosis be cured?

Currently, there is no cure for pulmonary fibrosis. Treatment focuses on managing symptoms, slowing disease progression, and improving overall quality of life. Research into potential treatments and therapies for pulmonary fibrosis is ongoing.

How does pulmonary fibrosis progress?

Pulmonary fibrosis is characterised by progressive scarring and stiffening of the lung tissue, which impairs lung function over time. As the disease progresses, individuals may experience worsening symptoms such as shortness of breath, cough, fatigue, and decreased exercise tolerance. In advanced stages, respiratory failure and complications such as pulmonary hypertension may occur.

What is the life expectancy for people who have pulmonary fibrosis?

The life expectancy for people with pulmonary fibrosis varies depending on factors such as the underlying cause, the severity of lung involvement, and the individual's overall health. On average, the prognosis for pulmonary fibrosis ranges from 3 to 5 years after diagnosis, but some individuals may live longer, especially with proper treatment and management.

Can pulmonary fibrosis be prevented?

While not all cases of pulmonary fibrosis can be prevented, there are steps individuals can take to reduce their risk, such as avoiding smoking and minimising exposure to environmental toxins and pollutants. Early detection and prompt management of respiratory symptoms and underlying medical conditions can also help reduce the risk of developing pulmonary fibrosis.

Who treats pulmonary fibrosis?

Pulmonary fibrosis is typically managed by a multidisciplinary team of healthcare professionals, including pulmonologists (lung specialists), respiratory therapists, nurses, and other specialists such as rheumatologists or radiologists. Treatment may also involve physical therapists, nutritionists, and social workers to address various aspects of the condition and provide comprehensive care for individuals with pulmonary fibrosis.

Reviewed by Dr. Sandeep Garg, Senior Consultant - Pulmonology on 29-Apr-24.