Skip to main content
RespiratoryICD-10: J84.1Affects approximately 13-20 per 100,000 people worldwide; IPF incidence is rising

Pulmonary Fibrosis

Pulmonary fibrosis is a chronic, progressive lung disease in which the tissue deep in the lungs becomes scarred (fibrotic), making the lungs thick and stiff. This scarring makes it increasingly difficult to breathe and prevents adequate oxygen from reaching the bloodstream. When no identifiable cause is found, the condition is called idiopathic pulmonary fibrosis (IPF), which is the most common and severe form.

Symptoms

Progressive shortness of breath, especially during physical activity
Persistent dry, hacking cough
Fatigue and generalized weakness
Unexplained weight loss
Aching muscles and joints
Clubbing (widening and rounding) of the fingertips or toes
Shallow, rapid breathing

Causes

  • Long-term exposure to environmental toxins such as asbestos, silica, or coal dust
  • Radiation therapy to the chest for cancer treatment
  • Certain medications including chemotherapy drugs, heart medications, and some antibiotics
  • Autoimmune diseases such as rheumatoid arthritis, scleroderma, or lupus
  • Idiopathic (unknown cause) in many cases

Risk Factors

  • Age between 50 and 70 years
  • History of cigarette smoking
  • Occupational exposure to industrial dusts and chemicals
  • Family history of pulmonary fibrosis
  • Gastroesophageal reflux disease (GERD)
  • Male sex (slightly higher incidence)

Diagnosis

  • High-resolution computed tomography (HRCT) scan of the chest
  • Pulmonary function tests (spirometry, diffusion capacity)
  • Pulse oximetry and arterial blood gas analysis
  • Surgical lung biopsy in uncertain cases
  • Bronchoalveolar lavage (BAL) to analyze lung fluid

Treatment

  • Antifibrotic medications (pirfenidone, nintedanib) to slow disease progression
  • Supplemental oxygen therapy to maintain blood oxygen levels
  • Pulmonary rehabilitation including exercise training and breathing techniques
  • Lung transplantation for advanced, end-stage disease
  • Treatment of underlying causes such as GERD or autoimmune conditions
  • Palliative care for symptom management in advanced stages

Prevention

  • Avoid exposure to occupational and environmental lung irritants
  • Wear appropriate respiratory protective equipment in dusty or toxic environments
  • Quit smoking and avoid secondhand smoke exposure
  • Get vaccinated against influenza and pneumococcal disease
  • Manage gastroesophageal reflux disease to prevent aspiration

When to See a Doctor

  • You experience progressively worsening shortness of breath
  • A persistent dry cough lasts more than 3-4 weeks without improvement
  • You notice fingertip or toe clubbing
  • You have known occupational exposure to dusts or toxins and develop respiratory symptoms

Frequently Asked Questions

Related Conditions

Have questions about Pulmonary Fibrosis?

Ask Mother Nature AI for personalized, evidence-based guidance.

Ask about Pulmonary Fibrosis

Medical Disclaimer: This content is for educational and informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment. Always consult a qualified healthcare provider.

Content reviewed against peer-reviewed medical literature and clinical guidelines. Read our editorial standards.