Idiopathic Pulmonary Fibrosis (IPF)

Etiology

  • Idiopathic
  • Radiographic and histologic findings of Usual Interstitial Pneumonia (UIP)

Risk Factors

  • Age> 60
  • Family hx, smoking

Symptoms

  • Sub-acute and progressive sob and doe
  • Sometimes acute "flairs" of symptoms that are idiopathic (evaluation to exclude infection and/or volume overload), leading to superimposed abrupt episodes of clinical decline
  • Progressive dry cough
  • Median survival 3y

Physical Exam Findings

  • Characteristic dry/velcro crackles on exam
  • Sometimes clubbing, cyanosis

Tests

Links