Relevant Physiology

Signs and Symptoms

• Clinical presentation (HPI):
  • 50-70 yr old, Older Adult > 65 (RF)
  • SOB, dry cough, exercise intolerance with relentless progression

Etiology

Unknown

Prognosis

• Median survival time 3-5 yrs

• Patient often die of !Hypoxemic/Type I RF

• GAP: Gender, Age, PFT (FVC and DLCO) for IPF mortality

  GAP Index for Idiopathic Pulmonary Fibrosis (IPF) Mortality

  • Male, older, poorer lung function (FVC, DLCO) = poor prognosis

Investigation

Tests

• Rule out other causes
• CXR - Chest X ray → Lower Lobe Disease
• HRCT - High Resolution Thoracic CT showing UIP Patterns
• Surgical Lung biopsy ONLY if HRCT is not conclusive

Decision Process

Management