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.

Treatment