VIDA LAB by Rowan Albritton
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respiratory

Pulmonary Fibrosis

A progressive lung disease where lung tissue becomes scarred and stiff, making breathing increasingly difficult.

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In this report
  1. Overview
  2. Symptoms
  3. Diagnosis
  4. Treatments
  5. Medical Resources
  6. Your Care Plan

Overview

Pulmonary fibrosis is a lung disease characterized by scarring (fibrosis) of the lung tissue. The scar tissue is thick and stiff, making it difficult for the lungs to expand and for oxygen to pass into the bloodstream. The disease is progressive and currently irreversible.

There are many causes of pulmonary fibrosis, including autoimmune diseases (rheumatoid arthritis, scleroderma), environmental exposures (asbestos, silica, bird droppings, mold), radiation therapy, certain medications, and infections. When no cause is identified, it's called idiopathic pulmonary fibrosis (IPF), the most common and severe form.

PF affects about 200,000 people in the United States and is more common in men and people over 60. The disease is progressive, and the rate of progression varies. While there is no cure, antifibrotic medications can slow progression, and lung transplant is an option for advanced disease. Early diagnosis and treatment are important to preserve lung function.

Symptoms

  • Shortness of breath, especially with exertion
  • Chronic dry, hacking cough
  • Fatigue
  • Unexplained weight loss
  • Aching muscles and joints
  • Clubbing — widening and rounding of the fingertips
  • Rapid, shallow breathing
  • Acute exacerbations — sudden, severe worsening of symptoms

Diagnosis

  1. High-resolution CT (HRCT) of the chest — the key imaging test; shows characteristic patterns of fibrosis (honeycombing, reticulation, traction bronchiectasis)
  2. Pulmonary function tests — show restrictive pattern (reduced lung volumes) and reduced diffusion capacity
  3. Blood tests — autoimmune markers (ANA, RF, anti-Scl-70) to identify connective tissue disease-associated PF
  4. Bronchoscopy with bronchoalveolar lavage — to rule out infection or other conditions
  5. Lung biopsy — occasionally needed if imaging is inconclusive
  6. Six-minute walk test — to assess functional capacity and oxygen needs

A pulmonologist specializing in interstitial lung disease makes the diagnosis.

Treatments

  • Antifibrotic medications — pirfenidone and nintedanib; slow disease progression in IPF
  • Immunosuppressive medications — for autoimmune-associated PF (corticosteroids, mycophenolate, rituximab)
  • Oxygen therapy — to maintain blood oxygen levels; improves quality of life and survival
  • Pulmonary rehabilitation — exercise training, education, and support
  • Lung transplant — for advanced disease; can be life-saving
  • Treating underlying cause — managing autoimmune disease, removing environmental exposure
  • Vaccinations — annual flu, pneumococcal, COVID-19
  • Lifestyle — smoking cessation, avoiding environmental exposures, maintaining activity
  • Acute exacerbation management — hospitalization, high-dose steroids, oxygen support
  • Palliative care — for symptom management in advanced disease

Your Care Plan

A step-by-step guide to navigating your condition, from finding the right doctors to advocating for the care you deserve.

Step 1: Doctors to See

Start with: Your primary care physician for initial evaluation.

Then seek: A pulmonologist who specializes in interstitial lung disease (ILD).

Build your team:

  • Pulmonologist (ILD specialist) — for disease management
  • Rheumatologist — if associated with autoimmune disease
  • Respiratory therapist — for oxygen and breathing techniques
  • Cardiologist — as PF can affect the heart
  • Lung transplant team — for advanced disease
  • Mental health professional — for anxiety and depression

Step 2: Advocate for Yourself

If you have progressive shortness of breath and chronic dry cough, ask for a high-resolution CT scan — don't let it be dismissed as aging or 'just a cough.' If diagnosed with PF, ask about antifibrotic medications — they can slow progression. If you're told nothing can be done, seek a second opinion at a specialized ILD center. If your oxygen is low, use supplemental oxygen as prescribed — it improves survival. If disease is advanced, ask about lung transplant evaluation — age alone shouldn't disqualify you.

Step 3: Your Action Plan

  1. See your doctor if you have chronic shortness of breath or dry cough.
  2. Get a high-resolution CT scan of the chest.
  3. Get pulmonary function tests to assess severity.
  4. See a pulmonologist who specializes in ILD.
  5. Identify the cause if possible — autoimmune disease, environmental exposure, or medication.
  6. Start treatment as prescribed — antifibrotics or immunosuppressants.
  7. Get evaluated for oxygen therapy if needed.
  8. Discuss lung transplant if disease is advanced.

Important: This report is educational information, not medical advice. Always consult a qualified healthcare professional for diagnosis and treatment decisions.

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