Abstract
Hamman-Rich syndrome, also known as acute interstitial pneumonia, is a rare and fulminant form of idiopathic interstitial lung disease. It should be considered as a cause of idiopathic acute respiratory distress syndrome. Confirmatory diagnosis requires demonstration of diffuse alveolar damage on lung histopathology. The main treatment is supportive care. It is not clear if glucocorticoid therapy is effective in acute interstitial pneumonia. We report the case of a 77-year-old woman without pre-existing lung disease who initially presented with mild upper respiratory tract infection and then progressed to rapid onset of hypoxic respiratory failure similar to acute respiratory distress syndrome with unknown etiology. Despite glucocorticoid therapy, she did not achieve remission and expired after 35 days of hospitalization. The diagnosis of acute interstitial pneumonia was supported by the histopathologic findings on her lung biopsy.
Highlights
Acute interstitial pneumonia (AIP) or Hamman Rich syndrome is a rare and fulminant form of lung injury, originally described by Hamman and Rich in 1935 [1]. It is an interstitial lung disease characterized by rapid onset of respiratory failure, similar to acute respiratory distress syndrome (ARDS) with diffuse alveolar damage (DAD) on lung biopsy specimens
AIP is characterized by the rapid development of acute respiratory failure in a previously healthy individual without a history of lung disease
The diagnosis was confirmed by the histological finding of DAD pattern seen on lung biopsy specimen
Summary
Acute interstitial pneumonia (AIP) or Hamman Rich syndrome is a rare and fulminant form of lung injury, originally described by Hamman and Rich in 1935 [1]. It is an interstitial lung disease characterized by rapid onset of respiratory failure, similar to acute respiratory distress syndrome (ARDS) with diffuse alveolar damage (DAD) on lung biopsy specimens. AIP is characterized by the rapid development of acute respiratory failure in a previously healthy individual without a history of lung disease. It is not associated with cigarette smoking and occurs with roughly equal frequency in men and women. The disease progression was not reversed by either mechanical ventilation or intravenous steroid therapy, and the patient expired after 35 days of hospitalization
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