Pulmonology

Idiopathic pulmonary fibrosis (IPF)

A severe progressive lung disease

Dr. Karim Benhaddou

Pulmonologist

6 min read
Idiopathic pulmonary fibrosis (IPF)

Progressive lung scarring

IPF is a chronic interstitial pneumonia of unknown cause. Mainly affects men >60 years. Median survival without treatment: 3-5 years.

Symptoms

Progressive exertional dyspnea, dry cough, Velcro-like crackles on auscultation, clubbing.

Diagnosis

High-resolution chest CT (typical UIP pattern), pulmonary function tests (restrictive pattern, decreased DLCO), multidisciplinary discussion. Lung biopsy in selected cases.

Treatments

  • Pirfenidone, nintedanib: antifibrotics, slow functional decline
  • Long-term oxygen if hypoxemia
  • Pulmonary rehabilitation: improves quality of life
  • Lung transplant: only curative option for eligible patients

Management

Vaccinations (flu, pneumococcus, COVID), avoid respiratory infections, supportive care, palliative care.

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