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Key Features

  • Known clinically as Hamman-Rich syndrome

Clinical Findings

  • Wide age range, including many young patients

  • Acute onset of dyspnea followed by rapid development of respiratory failure

  • Preceding viral syndrome is reported by 50% of patients

  • Clinical course is indistinguishable from idiopathic acute respiratory distress syndrome (ARDS)

Diagnosis

  • Radiograph shows diffuse bilateral airspace consolidation

  • High-resolution CT scan shows areas of ground-glass attenuation

  • Biopsy resembles the organizing phase of diffuse alveolar damage, with fibrosis and minimal collagen deposition

  • Pathologically similar to usual interstitial pneumonia but more homogeneous, and honeycombing is typically absent

Treatment

  • Supportive care including mechanical ventilation is critical

  • Benefit of specific therapies is unclear

  • 50–90% of patients die within 2 months

  • Course is not progressive if patient survives

  • Lung function may be permanently impaired in survivors

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