Correct!
2. Acute interstitial pneumonia (Hamman Rich syndrome)

In 1935 Hamman and Rich (2) described a disorder of rapidly progressive pneumonia that was not infectious or tuberculosis. This rare syndrome is characterized by rapidly progressive pulmonary fibrosis, leading to frequent confusion with idiopathic pulmonary fibrosis (3). In fact, the eponym Hamman Rich syndrome became synonymous with idiopathic pulmonary fibrosis despite clear differences in clinical presentation, radiography, pathology, and survival. In 1986, Katzenstein described eight patients with acute respiratory failure of unknown etiology. On biopsy, organizing diffuse alveolar damage was present in all specimens. Katzenstein coined the phrase acute interstitial pneumonitis (AIP) to distinguish it from the fibroproliferative stage of the acute respiratory distress syndrome (ARDS), which has an identical pathology. Since then, little progress into understanding this disease has been made. Treatment with corticosteroids is usually recommended although data supporting their use is weak.

Our patient was begun on corticosteroids but had little clinical improvement. About three weeks in to hospitalization his oxygenation not improving when he had a sudden clinical deterioration with dyspnea and hypoxia. The thoracic CT scan was repeated (Figure 3).

Figure 3. Representative views from repeat thoracic CT scan.

What new finding is present on the repeat thoracic CT scan? (Click on the correct answer to proceed to the fifth and final panel)

  1. Development of worsening ground glass opacities
  2. Diffuse nodular disease
  3. Increasing pulmonary fibrosis
  4. Pneumothorax
  5. Pulmonary embolus

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