Pulmonary Fibrosis

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Pulmonary Fibrosis

Pulmonary fibrosis is a chronic, progressive lung disease characterized by scarring (fibrosis) of the lung tissue. This scarring thickens and stiffens the walls of the lungs, making it increasingly difficult for oxygen to pass into the bloodstream and for the lungs to expand normally.

🔍 What Happens in Pulmonary Fibrosis?

  • The alveoli (air sacs) become damaged and are gradually replaced by scar tissue.
  • Over time, this leads to reduced lung capacity, poor oxygen exchange, and progressive shortness of breath.

📋 Key Symptoms

  • Shortness of breath, especially with exertion
  • Chronic dry cough
  • Fatigue
  • Unexplained weight loss
  • Clubbing (widening and rounding of fingertips/toes)
  • Chest discomfort

🧪 Causes of Pulmonary Fibrosis

1. Idiopathic Pulmonary Fibrosis (IPF)

  • The most common and serious form
  • Cause is unknown
  • Typically affects people aged 50–70

2. Secondary Pulmonary Fibrosis

Caused by identifiable factors:

  • Autoimmune diseases (e.g., rheumatoid arthritis, scleroderma, lupus)
  • Occupational/environmental exposure (asbestos, silica dust, metal dust)
  • Radiation therapy to the chest
  • Certain medications (e.g., chemotherapy drugs, antibiotics like nitrofurantoin)
  • Infections (e.g., tuberculosis, COVID-19)
  • Genetic conditions

🩺 Diagnosis

  • High-resolution CT (HRCT) scan: Shows characteristic scarring pattern
  • Pulmonary function tests: Show reduced lung volume and gas exchange
  • Blood tests (for autoimmune markers or underlying causes)
  • Lung biopsy (sometimes needed for a definitive diagnosis)
  • Oxygen saturation monitoring
  • 6-minute walk test (assess exercise tolerance and oxygen needs)

💊 Treatment Options

There is no cure, but treatments can help slow progression and manage symptoms.

Medications

  • Antifibrotic drugs:
    • Pirfenidone (Esbriet)
    • Nintedanib (Ofev)
  • Corticosteroids and immunosuppressants (in non-idiopathic types)

Supportive Care

  • Oxygen therapy
  • Pulmonary rehabilitation
  • Vaccinations (flu, pneumonia)
  • Nutritional support

Advanced Treatments

  • Lung transplantation (in eligible, severely affected patients)

🔄 Prognosis

  • Pulmonary fibrosis is progressive and potentially fatal.
  • In IPF, median survival is about 3–5 years after diagnosis without treatment.
  • Early detection, lifestyle changes (e.g., quitting smoking), and newer antifibrotic drugs can slow progression and improve quality of life.

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