Avian pulmonary proteinosis: six cases and a review of the literature.

Abstract

Pulmonary alveolar proteinosis (PAP) is a disease of surfactant clearance in which functional abnormalities in alveolar macrophages lead to accumulation of surfactant within alveoli in mammals. Histologic examination of 6 avian autopsies, including 4 chickens, a turkey, and a cockatiel, revealed accumulation of hypereosinophilic densely arrayed lamellar material in the lungs that was magenta by periodic acid-Schiff stain and diastase resistant. Transmission electron microscopy of the proteinaceous material in 2 cases demonstrated alternating electron-dense and electron-lucent lamellae that formed whorls and had a regular periodicity of 6-14 nm, consistent with pulmonary surfactant. Given the anatomic differences between avian and mammalian lungs, we designated the presented condition "pulmonary proteinosis," which can be observed as both an incidental finding or, when severe, may be a contributing factor to death through respiratory failure.