What is the most likely diagnosis?
- 40 year-old with dry cough and shortness of breath
Contrast-enhanced axial CT scan of chest
- Hemophilia
- Usual Interstitial Pneumonia
- Alveolar Proteinosis
- Silicosis
- Lymphangiomyomatosis
Additional image - Contrast-enhanced axial CT scan of chest at a higher level
Additional Images
Contrast-enhanced axial CT scan of chest
Answer:
3. Alveolar Proteinosis
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Alveolar Proteinosis
General Considerations
- Rare disorder of unknown etiology
- Alveoli are filled with PAS-positive proteinaceous material derived from surfactant phospholipids and proteins
- Males are affected 4X more than females; the disease is most common from 20-50 years of age
- It may be primary or secondary (to pulmonary infections, inhalation of silica or insecticides and hematologic malignancies)
- Diagnosis is usually made by lung biopsy