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Pulmonary Fibrosis (Idiopathic Pulmonary Fibrosis)

A chronic lung disease that progressively worsens as lung tissue is replaced by scar tissue (fibrous tissue) and stiffens.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Göğüs Hastalıkları department. Book Appointment →

What is Pulmonary Fibrosis (Idiopathic Pulmonary Fibrosis)?

Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive disease characterized by the development of progressive fibrosis (scar tissue accumulation) in lung tissue for unknown reasons. The fibrous tissue settles between the alveoli and prevents the passage of gases into the blood.

IPF is mostly seen in men over the age of 50. A 'honeycomb' appearance and basal subpleural reticular opacities are characteristic on HRCT. Median survival from diagnosis is 3-5 years.

Antifibrotic medications (pirfenidone, nintedanib) can slow the progression of the disease but cannot reverse it. Lung transplantation is the only curative option in suitable candidates.

Symptoms

Slowly progressive shortness of breath, increasing with exercise
Dry, persistent cough
Fatigue and weight loss
Clubbing of the fingertips
'Velcro'-type coarse rales in respiratory sounds (at the lung base)
Cyanosis due to low oxygen (advanced stage)
Progressively decreasing exercise capacity

Risk Factors

Advanced age (50-70 years)
Male sex
Smoking
Gastroesophageal reflux
Family history (familial IPF — MUC5B mutation)
Exposure to dust (asbestos, metal dust)
Viral infections (EBV, etc.)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When progressive shortness of breath and dry cough occur together
  • When widespread infiltrations are detected on lung X-ray
  • Emergency presentation when an exacerbation (acute worsening) develops
  • Referral to a transplant center for lung transplantation evaluation
  • Periodic desaturation testing for oxygen need

Treatment Methods

01
Pirfenidone or nintedanib (antifibrotic drugs — slow progression)
02
Aggressive treatment of gastroesophageal reflux
03
Oxygen support (long-term oxygen therapy as needed)
04
Pulmonary rehabilitation
05
Influenza and pneumococcal vaccination
06
Lung transplantation (only curative option in suitable candidates)

Which Department to Visit?

You can visit our Göğüs Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Göğüs Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.