acute interstitial pneumonia; Hamman-Rich syndrome; idiopathic alveolar fibrosis; rapidly progressive pulmonary fibrosis (IAF)

Introduction

diagnosis of exclusion

Etiology

• patients lack risk factors for ARDS

Pathology

• diffuse alveolar damage

Clinical manifestations

• acute onset, days to weeks
• fulminant respiratory failure (ARDS)*
• nail clubbing
• early death

* clinically, pathologically & radiographically indistinguishable from ARDS

Radiology

• chest X-ray
• chest CT
  • diffuse ground glass appearance with consolidation

Complications

• short-term mortality is 50%

Management

• glucocorticoids are used, although evidence of benefit is anecdotal
• intensive care unit
  • mechanical ventilation
    • low tidal volume (6 mL/kg of ideal body weight)
• patients who recover may relapse or develop chronic interstitial lung disease

More general terms

• interstitial pneumonia
• pulmonary fibrosis