Pulmonary Fibrosis
Advanced care for pulmonary fibrosis and interstitial lung diseases. Our pulmonology specialists provide comprehensive treatment to manage symptoms, slow disease progression, and improve quality of life.
Understanding Pulmonary Fibrosis
Pulmonary fibrosis (PF) is a progressive lung disease that causes scarring (fibrosis) of the lung tissue. This scarring makes the lungs stiff and thick, making it increasingly difficult to breathe and get enough oxygen into the bloodstream. As the disease progresses, patients experience worsening shortness of breath and decreased exercise tolerance.
Pulmonary fibrosis affects approximately 5 million people worldwide, with about 100,000 new cases diagnosed annually in the US alone. While there is no cure, advances in treatment have significantly improved outcomes and quality of life for patients.
Did You Know? The average survival rate for pulmonary fibrosis is 3-5 years from diagnosis, but early detection and treatment can significantly extend this timeline.
Types of Pulmonary Fibrosis
Idiopathic PF (IPF)
Most common type, cause unknown. Accounts for 50% of all PF cases. Typically affects adults 50+ years.
Occupational PF
Caused by exposure to asbestos (asbestosis), silica dust (silicosis), coal dust, or metal dusts.
Drug-Induced PF
Caused by medications like amiodarone, methotrexate, nitrofurantoin, or chemotherapy drugs.
Autoimmune-Related PF
Associated with rheumatoid arthritis, scleroderma, lupus, or Sjögren's syndrome.
Post-COVID PF
Lung scarring following severe COVID-19 infection, increasingly common complication.
Familial PF
Genetic form affecting two or more family members. Linked to mutations in TERT, TERC, SFTPC genes.
Common Symptoms
Symptoms typically develop gradually and worsen over time:
Shortness of Breath
Initially with exertion, later at rest
Dry Cough
Persistent, non-productive cough
Fatigue
Extreme tiredness and weakness
Clubbing
Fingertips widen and round
Weight Loss
Unexplained appetite loss
Muscle/Joint Pain
Generalized body aches
Causes & Risk Factors
Environmental Exposures
- Asbestos fibers
- Silica dust (mining, construction)
- Coal dust
- Hard metal dusts (cobalt, tungsten)
- Bird droppings (bird fancier's lung)
- Mold and mildew
Medical & Lifestyle Factors
- Smoking (increases risk 2-3x)
- Autoimmune diseases
- Gastroesophageal reflux (GERD)
- Viral infections (EBV, hepatitis C)
- Radiation therapy to chest
- Family history of PF
- Age (most common 50-70 years)
Diagnostic Process
- High-Resolution CT Scan (HRCT): Gold standard for diagnosis. Shows characteristic "honeycombing" and ground-glass opacities.
- Pulmonary Function Tests (PFTs): Measures lung capacity and gas exchange. Shows restrictive pattern.
- 6-Minute Walk Test: Assesses exercise capacity and oxygen desaturation.
- Blood Tests: Autoimmune panels, genetic testing, and biomarkers.
- Bronchoscopy: Transbronchial biopsy or bronchoalveolar lavage.
- Surgical Lung Biopsy: Video-assisted thoracoscopic surgery (VATS) for definitive diagnosis.
When to Seek Emergency Care
Seek immediate medical attention if you experience:
- Sudden worsening of shortness of breath
- Acute respiratory distress
- Coughing up blood (hemoptysis)
- Blue or gray skin/lips (cyanosis)
- Chest pain or tightness
- Fever with worsening cough (possible infection)
- Sudden confusion or altered mental status
Treatment Options
Medical Treatments
Antifibrotic Medications
Pirfenidone (Esbriet) and Nintedanib (Ofev) — slow disease progression by 50%
Oxygen Therapy
Improves quality of life, reduces shortness of breath, prevents complications
Immunosuppressants
Mycophenolate mofetil, Azathioprine, Rituximab (for autoimmune-related PF)
Corticosteroids
Prednisone for acute exacerbations or inflammatory PF types
Antacids (GERD Treatment)
Proton pump inhibitors to prevent micro-aspiration
Pulmonary Rehabilitation
Exercise training, breathing techniques, nutrition counseling
Surgical & Advanced Options
- Lung Transplantation: Single or double lung transplant for eligible patients
- Extracorporeal Life Support (ECLS): Bridge to transplant
- Photopheresis: For certain types of PF
Lifestyle & Supportive Care
- Smoking cessation
- Pulmonary rehabilitation
- Nutritional support
- Vaccinations (flu, pneumonia, COVID)
- Palliative care integration
- Support groups
Prevention & Management Tips
Why Choose Our Pulmonary Center?
Comprehensive interstitial lung disease care
ILD Specialists
Expert pulmonologists
Advanced Imaging
High-resolution CT
Antifibrotic Therapy
Latest FDA-approved drugs
Lung Transplant Program
Affiliated transplant center
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