DIAGNOSIS

SYMPTOMS

  • Prominent diffuse crackles1
  • Severe exertional dyspnea that develops over a short period of time1
  • Median time from first symptom to presentation is 3 weeks1
  • Fever, cough, shortness of breath that increase in severity over 7-14 days, progressing to respiratory failure4

DIAGNOSTIC TESTS

Pulmonary function tests1

  • Restrictive pattern
  • Reduced DLco

HRCT5

  • Features: Exudative phase shows ground-glass opacities, airspace consolidation; organizing phase shows bronchial dilatation, architectural distortion
  • Distribution: Lower lung predominance, symmetric, bilateral

Surgical lung biopsy is typically used to diagnose AIP4

  • Histopathology pattern shows diffuse alveolar damage5
    • Exudative early phase shows hyaline membranes and diffuse alveolar infiltration by lymphocytes5
    • Proliferating phase shows alveolar wall thickening due to fibrosis and pneumocyte hyperplasia5

Chest X-ray1

  • Most common findings on chest X-ray are bilateral airspace opacification with air bronchograms
  • The distribution is often patchy, with sparing of the costophrenic angles

See also

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Interstitial lung disease in systemic sclerosis with a focus on chest CT

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