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IDIOPATHIC PULMONARY FIBROSIS

Idiopathic pulmonary fibrosis is the progressive parenchymal scarring & loss of pulmonary function due to unidentifiable cause of lung injury.

It is a specific form of chronic fibrosing interstitial pneumonia with the histopathologic characteristics of usual interstitial pneumonia.

Approximately 5% of patients are asymptomatic. Common signs and symptoms are exertional dyspnea, nonproductive cough, finger clubbing and bilateral inspiratory crackles.

Follow Up

  • Follow-up is recommended every 3 months for patients with rapid disease progression or health status deterioration
  • Six-month intervals for follow-up is recommended for patients with steadily progressing disease
  • Follow-up every 6 months is recommended for patients with stable disease & annual follow-up for patients with stable disease after 1 year
  • Patients with ongoing pulmonary rehabilitation should be reassessed at 6- or 12-month intervals
  • Pulse oximetry measurement at rest & during exertion is recommended during follow-up (3-6 month intervals) to monitor for oxygenation status
    • Oxygen desaturation of <88% prompts supplemental oxygenation 
  • Screening for complications (pulmonary hypertension, pulmonary embolism, coronary artery disease, lung cancer) may be done every follow-up
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