A72-14 From Ground Glass to Whole Lung Lavage: Unmasking Pulmonary Alveolar Proteinosis (PAP) Misdiagnosed as Recurrent Pneumonia
Abstract
Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by accumulation of surfactant material within alveoli due to impaired clearance by alveolar macrophages. The material is periodic acid-Schiff (PAS)-positive, composed of phospholipids and apoproteins, with minimal inflammation and preserved lung architecture. Clinically, patients present with progressive dyspnea, cough, and hypoxemia. Imaging often reveals bilateral ground-glass opacities (GGOs) with interlobular septal thickening, creating a “crazy-paving” pattern. PAP may be autoimmune, secondary, or congenital. Autoimmune PAP, mediated by anti-GM-CSF antibodies, accounts for most cases, whereas secondary PAP is associated with hematologic malignancies, infections, or occupational exposures. Whole-lung lavage (WLL) remains the mainstay of treatment.
A 48-year-old man with hypertension and remote sleeve gastrectomy presented with fever, cough, and dyspnea. Chest imaging showed bilateral GGOs consistent with multifocal pneumonia, and he was treated with levofloxacin and corticosteroids. Despite therapy, he remained hypoxemic and required home oxygen. Repeat CT revealed worsening GGOs with a crazy-paving pattern. Infectious and autoimmune evaluations were negative except for a positive ANA. Bronchoscopy with BAL was nondiagnostic, while repeat BAL and transbronchial biopsy showed alveoli filled with PAS-positive material, diagnostic of PAP. Anti-GM-CSF antibody was negative, suggesting secondary PAP.…
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