Pulmocrit 2024 Abstracts

Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease characterized by accumulation of lipoproteinaceous material within the alveoli due to defective surfactant clearance by macrophages.PAP may be congenital, primary and secondary.Primary PAP may be autoimmune or hereditary.Autoimmune PAP is characterized by circulating auto antibodies against granulocyte-macrophage colonystimulating factor (GM-CSF).Here, we present a case of 30-year-old lady with autoimmune PAP and severe hypoxemic respiratory failure with alveolar arteriolar gradient (A-a gradient) of 459 mmHg.Anti-GM-CSF antibody was positive.She underwent three sessions of sequential whole lung lavage (WLL) with 30.5 litres of warmed saline despite several challenges.Experience of managing such patients is limited only to case reports and case series.Milky effluent fluid with sedimentation was obtained during WLL and resulted in marked improvement in her symptoms and correction of respiratory failure with A-a gradient of 12.9 mmHg after three sessions of WLL.HRCT chest showed diffuse and widespread ground glass opacities with superimposed smooth interstitial thickening giving "crazy paving" pattern in both lungs at presentation followed with significant clearing of GGO's and interlobular septal thickening after three sessions of WLL.In conclusion, therapeutic WLL can be successfully performed as a definitive treatment for a patient with severe PAP as in our case.