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

Pulmonary Fibrosis

Also known as: Lung scarring, Scarring of the lungs, Lung fibrosis, Fibrotic lung disease, Idiopathic pulmonary fibrosis, IPF, Interstitial lung disease

Pulmonary fibrosis is a chronic, progressive lung disease in which the delicate tissue deep in the lungs becomes scarred and thickened (fibrotic). This scarring — often described simply as 'lung scarring' — makes the lungs stiff and stops them from expanding normally, so it becomes progressively harder to breathe and less oxygen reaches the bloodstream. It is one of a family of more than 200 interstitial lung diseases. When no cause can be identified, the condition is called idiopathic pulmonary fibrosis (IPF), the most common and most serious form. Pulmonary fibrosis is not cancer and is not the same as COPD, but it is serious and tends to worsen over time; modern antifibrotic medications can slow its progression, and the earlier it is caught, the more lung function can be preserved.

At a Glance

Common symptoms
Progressive shortness of breath, especially during physical activity (the hallmark symptom), Persistent dry, hacking cough that does not go away, Fatigue and generalized weakness
Main treatments
Antifibrotic medications (pirfenidone, nintedanib) to slow disease progression, Supplemental oxygen therapy to maintain blood oxygen levels, Pulmonary rehabilitation including exercise training and breathing techniques
Category
Respiratory · Affects approximately 13-20 per 100,000 people worldwide; IPF incidence is rising
See a doctor if
You experience progressively worsening shortness of breath

Symptoms

Progressive shortness of breath, especially during physical activity (the hallmark symptom)
Persistent dry, hacking cough that does not go away
Fatigue and generalized weakness
Unexplained weight loss and reduced appetite
Aching muscles and joints
Clubbing (widening and rounding) of the fingertips or toes
Shallow, rapid breathing
Crackling sounds in the lungs (heard by a doctor with a stethoscope)

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

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