Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease that causes irreversible scarring (fibrosis) and impairs lung function. Most patients present with exertional dyspnea and a persistent dry cough. Without treatment, IPF has a poor prognosis, with a median survival of 3–5 years from diagnosis.
What causes IPF?
The exact cause of IPF is unknown. Evidence suggests that repeated injury to the alveolar epithelium and abnormal repair processes drive fibrosis. Genetic predisposition, such as MUC5B promoter polymorphism and telomerase mutations, increases susceptibility, while environmental exposures, including smoking, air pollution, and microaspiration, also play a role.
What are the symptoms of IPF?
Patients with IPF commonly report:
- Progressive exertional dyspnea
- Dry cough
- Fatigue
Clinicians often detect fine inspiratory crackles and digital clubbing during physical examination. Pulmonary function tests typically reveal a restrictive pattern and reduced diffusing capacity for carbon monoxide.
How is IPF diagnosed?
Diagnosing IPF involves:
- Reviewing the patient’s clinical history
- Confirming a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT)
- Ruling out other causes, such as connective tissue disease (e.g., rheumatoid arthritis, rashes, skin changes) or environmental exposures
If imaging is inconclusive, consider lung biopsy.
A multidisciplinary review involving pulmonologists, radiologists, and pathologists is recommended to confirm the diagnosis.
Pulmonary function tests help monitor disease progression and response to therapy.
What are the treatment options for IPF?
Management of IPF includes antifibrotic agents, such as nintedanib and pirfenidone, which slow disease progression. Supportive care includes:
- Oxygen therapy
- Pulmonary rehabilitation
- Management of comorbidities
Lung transplantation may be considered for eligible patients.
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