Idiopathic pulmonary fibrosis (IPF)
A severe progressive lung disease
Dr. Karim Benhaddou
Pulmonologist
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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