Pulmonary Fibrosis: Treatment Options & Disease Care

Pulmonary Fibrosis Treatment

Pulmonary fibrosis (PF) is a long-term, progressive lung disease that results in scarring (fibrosis) of the lung tissue. The scarring complicates the functioning of the lungs. Though there is no treatment for the majority of pulmonary fibrosis types, treatments to retard the disease, treat symptoms, and enhance quality of life are available.

Best Treatment for Pulmonary Fibrosis

The optimal treatment for pulmonary fibrosis varies with the type, etiology, severity, and patient's individual health status. What follows is a brief overview of the most optimal and evidence-based therapies up to 2025, particularly for Idiopathic Pulmonary Fibrosis (IPF), the most frequent type. Best Treatment for Pulmonary Fibrosis:

Antifibrotic drugs (Optimal for delaying disease progression in IPF)

Nintedanib (Ofev)

• Mechanism: Tyrosine kinase inhibitor
• Benefits: Slows lung function decline, decreases exacerbations

Pirfenidone (Esbriet)

• Mechanism: Anti-inflammatory & antifibrotic
• Benefits: Slows progression of disease and can improve survival

Lung Transplantation (Best option for suitable patients with severe disease)

• Only treatment with a chance to cure.

Recommended for:

• Accelerating disease
• Extensive functional impairment
• Failure to respond to medication
• Survival rate after 5 years: ~50–60%.

Pulmonary Rehabilitation (Best for enhancing quality of life)

• Exercise training, breathing exercises, and education

Benefits:

• Improved stamina and breathing efficiency
• Fewer symptoms such as shortness of breath
• Enhanced emotional well-being

Oxygen Therapy (Best for those with low oxygen saturation)

• Used at rest, during exercise, or during sleep
• Prevents complications associated with hypoxia and enhances comfort

Treat the Underlying Cause (If PF is secondary)

If fibrosis is due to:

Autoimmune disease (e.g., RA, scleroderma) → Utilize immunosuppressants (e.g., mycophenolate, rituximab)
Environmental exposure → Remove exposure + steroids/immunosuppressants
Drug-induced PF → Withdraw offending drug + potential use of steroid

Experimental Therapies & Clinical Trials

• Gene therapy, cell therapy, and new antifibrotic drugs
• Could provide new hope to non-responding patients to current drugs

Pulmonary Fibrosis Medication Options

Below are the primary medication options for pulmonary fibrosis (PF), by disease type and function:

Antifibrotic Medications (First-line treatment for Idiopathic Pulmonary Fibrosis – IPF)

• These medications delay the worsening of fibrosis but are not curative.

Pirfenidone (Esbriet)

• Mechanism: Anti-inflammatory, antioxidant, and antifibrotic
• Dosage: 2403 mg/day (typically 3 tablets/day)
• Advantages: Slows lung function decline, possibly improves survival

Typical Side Effects:

• Nausea, diarrhea, tiredness
• Sensitivity to sun (photosensitivity)
• Elevation of liver enzymes

Nintedanib (Ofev)

• Action: Tyrosine kinase inhibitor (inhibits mechanisms causing fibrosis)
• Dose: 150 mg twice a day
• Benefits: Slows rate of decline in lung function and exacerbations

Common Side Effects:

• Diarrhea (most common)
• Nausea, vomiting
• Elevation of liver enzymes

Immunosuppressive Drugs

• Used in non-IPF forms of PF, particularly those due to autoimmune disorders (e.g., scleroderma, rheumatoid arthritis, lupus).

Prednisone

• Initial suppression of inflammation
• Weight gain, diabetes, osteoporosis

Azathioprine

• Maintenance therapy
• Nausea, liver problems, suppression of bone marrow

Mycophenolate mofetil (CellCept)

• Most common in scleroderma-ILD
• GI problems, risk of infection

Cyclophosphamide

• Used in severe cases
• Bladder toxicity, suppression of bone marrow

Methotrexate

• For RA-associated ILD
• Toxicity in liver, lung (rare)

Rituximab

• Biologic for autoimmune ILD
• Infusion reactions, risk of infection

Supportive Medications

• To treat symptoms or complications.
• Proton pump inhibitors (PPIs): GERD management (most common in PF)
• Antibiotics: Prevent/treat lung infections
• Diuretics: Treat heart failure-like symptoms (fluid overload)
• Antidepressants / Anxiolytics: Manage mental health in chronic illness

Medications Under Research / Trials

• Ziritaxestat (autotaxin inhibitor) – trials discontinued due to side effects
• Pamrevlumab – promising antifibrotic in phase 3 trials
• Thyroid hormone analogs, anti-CTGF drugs, cell-based therapies – under investigation.

How to manage pulmonary fibrosis?

Pulmonary fibrosis (PF) management requires a multidisciplinary approach aimed at slowing disease progression, symptom relief, enhancing quality of life, and managing complications. Below is an in-depth guide on effective pulmonary fibrosis management.

Medicines to Slow Disease Progression

In Idiopathic Pulmonary Fibrosis (IPF) and Progressive Fibrosing ILD:

• Nintedanib (Ofev) – Slows lung scarring and functional decline.
• Pirfenidone (Esbriet) – Suppresses inflammation and fibrosis.
• In Autoimmune-related or Secondary PF:
• Corticosteroids (e.g., Prednisone) – Short-term inflammation suppression.
• Immunosuppressants (e.g., Mycophenolate, Azathioprine, Methotrexate) – Long-term management.

Oxygen Therapy

• When to use: If oxygen saturation (SpOâ‚‚) is <88% at rest or on effort.
• Benefits: Enhances energy, decreases breathlessness, and avoids low oxygen (hypoxia) complications.

Pulmonary Rehabilitation

A supervised program typically 8–12 weeks in duration involves:

• Supervised exercise training
• Breathing exercises (such as pursed-lip breathing)
• Education (lung health, pacing techniques)
• Nutritional and psychological counseling
• Demonstrated to enhance stamina, decrease symptoms, and increase emotional well-being.

Lifestyle Changes

Stop Smoking

• Smoking exacerbates lung damage. Quitting is paramount.

Avoid Lung Irritants

• Limit exposure to dust, molds, chemicals, and pollutants.

Eat a Balanced Diet

• Avoid unintended weight loss or malnutrition.

Stay Active

• Gentle walking, yoga, or swimming can preserve muscle tone and endurance.

Get Vaccinated

• Influenza, COVID-19, and pneumococcal vaccines lower the risk of respiratory infections.

Monitor & Follow-Up

• Regular pulmonologist check-ups

Monitor:

• Lung function tests (PFTs or spirometry)
• Oxygen saturation levels
• Liver function (if taking antifibrotics)
• Symptoms (cough, breathlessness, fatigue)

Lung Transplantation

• Consider for advanced disease or accelerated progression in spite of treatment.
• Best for patients <65–70 years with fewer comorbidities.

Palliative Care & Symptom Management

• Centers on comfort, symptom control, and emotional support.
• Medications for cough, anxiety, or sleep disturbances may be helpful.

Mental Health Support

• Breathlessness and changes in life may lead to depression or anxiety.
• Counselling, support groups, or medication could be helpful.

Join Clinical Trials (Optional)

• Access new therapies and help advance knowledge.
• Numerous trials are examining new antifibrotics, biologics, and stem cell therapy.

Latest Treatment for Pulmonary Fibrosis

As of 2025, the most recent treatment avenues for pulmonary fibrosis (PF)—particularly idiopathic pulmonary fibrosis (IPF) and progressive fibrosing interstitial lung diseases (PF-ILDs)—are both available approved therapies and potential promising experimental ones. Here's the breakdown:

Most Recent Approved Therapies

Nintedanib (Ofev)

• Mechanism: Tyrosine kinase inhibitor
• Indication: IPF, systemic sclerosis-associated ILD, and other PF-ILDs

Most Recent Developments:

• Now more commonly utilized outside of IPF for progressive fibrosing ILDs
• New data supports early treatment for improved long-term outcomes

Pirfenidone (Esbriet)

• Mechanism: Anti-inflammatory and antifibrotic
• Use: IPF (not yet approved for non-IPF PF-ILDs but being used off-label)

New Developments:

• Combination therapy with Nintedanib under investigation for severe disease
• Adjusted formulations being investigated to minimize side effects

New Treatments in Trials & Novel Therapies

Pamrevlumab

• Type: Anti-CTGF monoclonal antibody
• Mechanism: Inhibits connective tissue growth factor (CTGF), a major inducer of fibrosis
• Status: Phase III clinical trials for IPF and systemic sclerosis-associated ILD
• Promise: Possible to slow fibrosis without gastrointestinal side effects of Ofev

ZEPHYR Program (BI 1015550) – PDE4B Inhibitor

• Company: Boehringer Ingelheim
• Mechanism: Anti-inflammatory and antifibrotic
• Status: Phase III trials for IPF and PF-ILD
• Advantage: Oral, new mechanism, potentially safer in comorbid patients

Thyroid Hormone Analogs (e.g., Sobetirome)

• Mechanism: Enhance alveolar repair and mitochondrial function
• Status: Early-stage trials
• Promise: Regenerative potential—enhances lung tissue healing, not merely control of fibrosis

Cell-Based Therapies (Mesenchymal Stem Cells – MSCs)

• Status: In trials worldwide
• Mechanism: Anti-inflammatory, regenerative effects

Gene Editing and Anti-Fibrotic RNA Therapies

Strategy: Target genes (such as MUC5B, TERT) associated with fibrosis
Example: Antisense oligonucleotides in development
Stage: Preclinical/early human trials

Pulmonary Fibrosis Cure Possibilities

To date, as of 2025, there is no cure for pulmonary fibrosis — particularly idiopathic pulmonary fibrosis (IPF). Nevertheless, therapeutic advances have markedly enhanced the capacity to delay disease progression, control symptoms, and prolong quality life years.

Pulmonary Fibrosis Lung Transplant

Lung transplantation is the only definitive (curative) treatment for severe pulmonary fibrosis, particularly idiopathic pulmonary fibrosis (IPF) or progressive fibrosing ILDs unresponsive to medical therapy.

Who Needs a Lung Transplant?

Candidates for lung transplant characteristically:

• Have advanced or worsening PF
• Develop worsening symptoms despite medications (e.g., nintedanib, pirfenidone)
• Have downward spiraling lung function (FVC <50–60%, DLCO <40%)
• Need oxygen at rest or low level of activity
• Are typically <65–70 years of age and medically suitable for extensive surgery
• Transplantation is being considered where the life expectancy is <2–3 years without it.

Pulmonary Fibrosis Home Treatment

Though pulmonary fibrosis (PF) cannot be treated at home, supportive care and lifestyle modification at home can have a great impact on comfort, symptom management, and quality of life. These home remedies work best when supplemented by medications and ongoing medical visits.

Breathing Techniques

Pursed-Lip Breathing

• Breathe in for 2 seconds through your nose
• Slowly exhale using pursed lips (blow out a candle) for 4 seconds
• Lessens breathlessness and saves energy

Diaphragmatic (Belly) Breathing

• Put one hand on your tummy
• Breathe in deeply so your belly goes up, not your chest
• Aids to fill lungs more effectively

Home Oxygen Therapy (if needed)

• Follow direction for use of supplemental oxygen (resting, sleeping, or activity)
• Keep equipment clean and dry
• Do not smoke or have open flames near oxygen
• Utilize a home pulse oximeter to check oxygen levels (aim for > 90%).

Physical Activity (Gentle Exercise)

• Walking, stretching, or yoga every day increases stamina and efficiency of lungs
• Avoid overdoing it — take it easy
• Take a rest break or use a walker if necessary

Recommended Activities:

• Tai chi
• Chair yoga
• Breath-focused meditation

Healthy Diet

• Eat small, frequent meals to prevent feeling bloated (which makes breathlessness worse)
• High-protein, nutrient-dense foods (e.g., lentils, eggs, nuts)
• Drink plenty of fluid
• Restrict salt if you have swelling or heart disease
• Prevent gas-producing foods if bloating increases breathlessness (e.g., cabbage, carbonated beverages).

Rest & Sleep Hygiene

• Raise the head of the bed if nighttime coughing is worse
• Use a humidifier if air is dry
• Avoid heavy meals and alcohol at night

Steer Clear of Lung Irritants

• Don't smoke or permit smoking in the household
• Use air purifiers or N95 masks when exposed to dust, smoke, or pollution
• Steer clear of strong chemical cleaners and perfumes

Mental Health Support

• Chronic illness may lead to anxiety or depression
• Practice mindfulness or breathing-based relaxation
• Stay socially connected with family, friends, or support groups
• Online PF support communities can minimize feelings of isolation

Home Safety Advice

• Install bathroom handrails
• Clear out pathways to avoid falls, if on oxygen or mobility aids
• Utilize nonslip mats and proper lighting

Monitor Symptoms and Progress

Maintain a daily health notebook:

• Shortness of breath rating
• Oxygen level readings
• Fatigue or sleep
• Coughing spells
• This assists your doctor in making adjustments in treatment more precisely.

Keep Current with Vaccines

• Flu vaccine (annually)
• Pneumococcal vaccine
• COVID-19 vaccine
• Others as indicated

Anti-fibrotic Drugs for Pulmonary Fibrosis

Anti-fibrotic medications are the standard treatment for Idiopathic Pulmonary Fibrosis (IPF) and are now used in other progressive fibrosing interstitial lung diseases (PF-ILDs) more frequently. These medications delay the progression of scarring within the lungs but do not treat current fibrosis.

Nintedanib 

Overview:

• Type: Tyrosine kinase inhibitor
• Mechanism: Inhibits signaling pathways involved in fibrosis (PDGF, FGF, VEGF)

Approved For:

• Idiopathic Pulmonary Fibrosis (IPF)
• Systemic Sclerosis-associated ILD
• Other progressive fibrosing ILDs

Dosage:

• 150 mg twice daily

Common Side Effects:

• Diarrhea (most common)
• Nausea, vomiting
• Liver enzyme increase (check LFTs frequently)
• Loss of hunger
• Can be taken with food to minimize GI side effects

Pirfenidone 

Overview:

• Type: Oral antifibrotic and anti-inflammatory medication
• Mechanism: Suppresses fibroblast activity and collagen synthesis

Approved For:

• Idiopathic Pulmonary Fibrosis (IPF)
• Used off-label in a few PF-ILDs

Dosage:

• Target dose: 2403 mg/day, administered in 3 doses with meals

Common Side Effects:

• Nausea, indigestion
• Fatigue
• Rash, photosensitivity (avoid sun)
• Liver enzyme increase
• Needs gradual increase in dose to reduce side effects

Why Choose GetWellGo for Pulmonary Fibrosis Treatment?

GetWellGo is regarded as a leading supplier of healthcare services. We help our foreign clients choose the best treatment locations that suit their needs both financially and medically.

We offer:

• Complete transparency
• Fair costs.
• 24 hour availability.
• Medical E-visas
• Online consultation from recognized Indian experts.
• Assistance in selecting India's top hospitals for Pulmonary Fibrosis treatment.
• Expert doctor with a strong track record of success
• Assistance during and after the course of treatment.
• Language Support
• Travel and Accommodation Services
• Case manager assigned to every patient to provide seamless support in and out of the hospital like appointment booking
• Local SIM Cards
• Currency Exchange
• Arranging Patient’s local food