Syndromes characterized by the progressive accumulation of surfactant protein in the alveoli.
Epidemiology
Etiology
Autoimmunity
Pathophysiology
Progressive surfactant accumulation in the alveoli interferes with gas exchange.
Clinical features
- Dyspnea
- Cough (dry or productive), hemoptysis
- Cyanosis
- Weight loss, fatigue, malaise
Diagnostics
- X-ray/CT chest shows a characteristic pattern of ground-glass opacity and reticular densities (crazy-paving pattern)
- Histopathologic findings would include granular, proteinaceous material within the alveoli with normal alveolar architecture.
