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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.

Supportive Therapy

  • Supportive measures in patients with acute exacerbation of idiopathic pulmonary fibrosis should include relief of dyspnea & provision of supplemental oxygen

Anti-Acid Therapy

  • Acid aspiration & gastroesophageal reflux/gastroesophageal reflux disease causing acute exacerbation of idiopathic pulmonary fibrosis may be treated with antacids (ie proton pump inhibitors, histamine-2 blocker receptor antagonists)
    • May help reduce the risk for microaspiration-associated lung injury 
  • Stress ulcer prophylaxis guidelines must be followed

Antibiotics

  • May be considered in idiopathic pulmonary fibrosis patients in acute exacerbation with radiographic findings of overlapping pneumonia
  • If the culture is negative, a 7-day course of antibiotic is recommended

Symptom Control for Cough & Dyspnea

  • Facial cooling with a fan & the use of opioids & anxiolytics may help alleviate refractory cough & dyspnea

Prevention of Venous Thromboembolism (VTE)

  • Venous thromboembolism is common in patients who were hospitalized due to acute exacerbation of idiopathic pulmonary fibrosis & routine measures should be employed

Vaccine

  • Influenza & pneumococcal vaccines should be offered to patients with idiopathic pulmonary fibrosis
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